Porphobilinogen (BioDeep_00000001667)

 

Secondary id: BioDeep_00000405227

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite BioNovoGene_Lab2019 Volatile Flavor Compounds


代谢物信息卡片


3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid

化学式: C10H14N2O4 (226.0953524)
中文名称: 胆色素原, 紫质胆素原
谱图信息: 最多检出来源 Homo sapiens(blood) 0.1%

分子结构信息

SMILES: C(CC(=O)O)c1c[nH]c(CN)c1CC(=O)O
InChI: InChI=1S/C10H14N2O4/c11-4-8-7(3-10(15)16)6(5-12-8)1-2-9(13)14/h5,12H,1-4,11H2,(H,13,14)(H,15,16)

描述信息

Porphobilinogen (PBG) is a pyrrole-containing intermediate in the biosynthesis of porphyrins. It is generated from aminolevulinate (ALA) by the enzyme ALA dehydratase. Porphobilinogen is then converted into hydroxymethylbilane by the enzyme porphobilinogen deaminase (also known as hydroxymethylbilane synthase). Under certain conditions, porphobilinogen can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).
Porphobilinogen is a pyrrole involved in porphyrin metabolism. -- Wikipedia; It consists of a pyrrole ring with acetyl, propionyl, and aminomethyl side chains; It is a key monopyrrolic intermediate in porphyrin, chlorophyll and vitamin B12 biosynthesis. Porphobilinogen is generated by the enzyme ALA dehydratase by combining two molecules of dALA together, and converted into hydroxymethyl bilane by the enzyme porphobilinogen deaminase. 4 molecules of porphobilinogen are condensed to form one molecule of uroporphyrinogen III, which is then converted successively to coproporphyrinogen III, protoporphyrin IX, and heme. Porphobilinogen is produced in excess and excreted in the urine in acute intermittent porphyria and several other porphyrias. [HMDB]. Porphobilinogen is found in many foods, some of which are strawberry guava, amaranth, parsnip, and ostrich fern.

同义名列表

8 个代谢物同义名

3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid; 5-(Aminomethyl)-4-(carboxymethyl)-1H-pyrrole-3-propanoic acid; 5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionic acid; 5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionate; porphobilinogen; PBG; Porphobilinogen; Porphobilinogen



数据库引用编号

22 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(0)

BioCyc(0)

PlantCyc(0)

代谢反应

34 个相关的代谢反应过程信息。

Reactome(10)

BioCyc(1)

WikiPathways(2)

Plant Reactome(0)

INOH(1)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(20)

PharmGKB(0)

4 个相关的物种来源信息

在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：

  • PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
  • NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
  • Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
  • Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。

点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。



文献列表

  • Yushen Wang, Junjie Wang, Liqing Chen, Xiaowei Meng, Xiaoxi Zhen, Yinpei Liang, Yuanhuai Han, Hongying Li, Bin Zhang. Identification and function analysis of yellow-leaf mutant (YX-yl) of broomcorn millet. BMC plant biology. 2022 Sep; 22(1):463. doi: 10.1186/s12870-022-03843-y. [PMID: 36167497]
  • Yuxuan Wang, Jinrong Yang, Qingxiu Chen, Junyi Su, Wen-Jing Shi, Lei Zhang, Chunli Xia, Jinwu Yan. Rotor-Tuning Boron Dipyrromethenes for Dual-Functional Imaging of Aβ Oligomers and Viscosity. ACS applied bio materials. 2022 06; 5(6):3049-3056. doi: 10.1021/acsabm.2c00335. [PMID: 35671477]
  • Maria Grostøl Ørnhøj Østergaard, Erland Jørn Erlandsen, Henrik Holm Thomsen, Else Randers. Peritoneal dialysis resulting in discontinuance of recurring attacks of acute intermittent porphyria: A case report. Seminars in dialysis. 2022 01; 35(1):93-95. doi: 10.1111/sdi.13025. [PMID: 34558740]
  • Hongxia Xu, Shankun Yao, Yuncong Chen, Changli Zhang, Shuren Zhang, Hao Yuan, Zhongyan Chen, Yang Bai, Tao Yang, Zijian Guo, Weijiang He. Tracking Labile Copper Fluctuation In Vivo/Ex Vivo: Design and Application of a Ratiometric Near-Infrared Fluorophore Derived from 4-Aminostyrene-Conjugated Boron Dipyrromethene. Inorganic chemistry. 2021 Dec; 60(24):18567-18574. doi: 10.1021/acs.inorgchem.1c01779. [PMID: 34826221]
  • Thomas W Fredrick, Manuel B Braga Neto, Daniel O Johnsrud, Michael Camilleri, Victor G Chedid. Turning Purple with Pain. The New England journal of medicine. 2021 08; 385(6):549-554. doi: 10.1056/nejmcps2105278. [PMID: 34347956]
  • Galina Guseva, Elena Antina, Mikhail Berezin, Svetlana Lisovskaya, Roman Pavelyev, Airat Kayumov, Olga Lodochnikova, Daut Islamov, Konstantin Usachev, Sergei Boichuk, Liliya Nikitina. Spectroscopic and In Vitro Investigations of Boron(III) Complex with Meso-4-Methoxycarbonylpropylsubstituted Dipyrromethene for Fluorescence Bioimaging Applications. Molecules (Basel, Switzerland). 2020 Oct; 25(19):. doi: 10.3390/molecules25194541. [PMID: 33023057]
  • Giuseppe Comentale, Rachele Manzo, Emanuele Pilato. Sars-Cov-2 interference in HEME production: is it the time for an early predictive biomarker?. Journal of molecular medicine (Berlin, Germany). 2020 08; 98(8):1053-1054. doi: 10.1007/s00109-020-01945-4. [PMID: 32601795]
  • Yon Namkoong, Jinrok Oh, Jong-In Hong. Electrochemiluminescent detection of glucose in human serum by BODIPY-based chemodosimeters for hydrogen peroxide using accelerated self-immolation of boronates. Chemical communications (Cambridge, England). 2020 Jul; 56(55):7577-7580. doi: 10.1039/d0cc03315d. [PMID: 32510098]
  • Sagar Agarwal, Amy R Simon, Varun Goel, Bahru A Habtemariam, Valerie A Clausen, Jae B Kim, Gabriel J Robbie. Pharmacokinetics and Pharmacodynamics of the Small Interfering Ribonucleic Acid, Givosiran, in Patients With Acute Hepatic Porphyria. Clinical pharmacology and therapeutics. 2020 07; 108(1):63-72. doi: 10.1002/cpt.1802. [PMID: 31994716]
  • Manisha Balwani, Eliane Sardh, Paolo Ventura, Paula Aguilera Peiró, David C Rees, Ulrich Stölzel, D Montgomery Bissell, Herbert L Bonkovsky, Jerzy Windyga, Karl E Anderson, Charles Parker, Samuel M Silver, Siobán B Keel, Jiaan-Der Wang, Penelope E Stein, Pauline Harper, Daphne Vassiliou, Bruce Wang, John Phillips, Aneta Ivanova, Janneke G Langendonk, Raili Kauppinen, Elisabeth Minder, Yutaka Horie, Craig Penz, Jihong Chen, Shangbin Liu, John J Ko, Marianne T Sweetser, Pushkal Garg, Akshay Vaishnaw, Jae B Kim, Amy R Simon, Laurent Gouya. Phase 3 Trial of RNAi Therapeutic Givosiran for Acute Intermittent Porphyria. The New England journal of medicine. 2020 06; 382(24):2289-2301. doi: 10.1056/nejmoa1913147. [PMID: 32521132]
  • Anis Cerovac, Alma Brigic, Dzenana Softic, Amna Barakovic, Semir Adzajlic. Uncontrolled Acute Intermittent Porphyria as a Cause of Spontaneous Abortion. Medical archives (Sarajevo, Bosnia and Herzegovina). 2020 Apr; 74(2):153-155. doi: 10.5455/medarh.2020.74.153-155. [PMID: 32577061]
  • José Bustos, Ledmar Vargas, Ricardo Quintero. Acute intermittent porphyria: A case report. Biomedica : revista del Instituto Nacional de Salud. 2020 03; 40(1):14-19. doi: 10.7705/biomedica.4767. [PMID: 32220159]
  • R Golla, A Mukherjee, R K Gone, H Singh, A K Pannu, V Suri, A Bhalla. Acute intermittent porphyria and anti-tuberculosis therapy. QJM : monthly journal of the Association of Physicians. 2020 Mar; 113(3):207-208. doi: 10.1093/qjmed/hcz289. [PMID: 31693148]
  • Karl E Anderson. Acute hepatic porphyrias: Current diagnosis & management. Molecular genetics and metabolism. 2019 11; 128(3):219-227. doi: 10.1016/j.ymgme.2019.07.002. [PMID: 31311713]
  • Diego Vera-Yunca, Irantzu Serrano-Mendioroz, Ana Sampedro, Daniel Jericó, Iñaki F Trocóniz, Antonio Fontanellas, Zinnia P Parra-Guillén. Computational disease model of phenobarbital-induced acute attacks in an acute intermittent porphyria mouse model. Molecular genetics and metabolism. 2019 11; 128(3):367-375. doi: 10.1016/j.ymgme.2018.12.009. [PMID: 30639045]
  • Elin Storjord, Jim A Dahl, Anne Landsem, Judith K Ludviksen, Marlene B Karlsen, Bård O Karlsen, Ole-L Brekke. Lifestyle factors including diet and biochemical biomarkers in acute intermittent porphyria: Results from a case-control study in northern Norway. Molecular genetics and metabolism. 2019 11; 128(3):254-270. doi: 10.1016/j.ymgme.2018.12.006. [PMID: 30583995]
  • Kevin J Guzman, Laxmi A Suthar. Axonal Polyneuropathy in a Man Treated for Pulmonary Cocci: A Case of Acute Intermittent Porphyria. The American journal of case reports. 2019 Sep; 20(?):1378-1381. doi: 10.12659/ajcr.917134. [PMID: 31530795]
  • Ulrich Stölzel, Manfred O Doss, Detlef Schuppan. Clinical Guide and Update on Porphyrias. Gastroenterology. 2019 08; 157(2):365-381.e4. doi: 10.1053/j.gastro.2019.04.050. [PMID: 31085196]
  • Yi He, Gong-Jian Fan, Cai-E Wu, Xiao-Hong Kou, Ting-Ting Li. Influence of illumination on the greening and relative enzyme activity of garlic puree. Journal of food biochemistry. 2019 07; 43(7):e12871. doi: 10.1111/jfbc.12871. [PMID: 31353726]
  • Mehmet M Tatari, Josiah D McCain, Jed C Cowdell. 28-Year-Old Woman With Severe Generalized Abdominal Pain. Mayo Clinic proceedings. 2019 07; 94(7):1334-1338. doi: 10.1016/j.mayocp.2018.10.031. [PMID: 31272576]
  • Makiko Yasuda, Lin Gan, Brenden Chen, Chunli Yu, Jinglan Zhang, Miguel A Gama-Sosa, Daniela D Pollak, Stefanie Berger, John D Phillips, Winfried Edelmann, Robert J Desnick. Homozygous hydroxymethylbilane synthase knock-in mice provide pathogenic insights into the severe neurological impairments present in human homozygous dominant acute intermittent porphyria. Human molecular genetics. 2019 06; 28(11):1755-1767. doi: 10.1093/hmg/ddz003. [PMID: 30615115]
  • María Barreda-Sánchez, Juan Buendía-Martínez, Guillermo Glover-López, Carmen Carazo-Díaz, María Juliana Ballesta-Martínez, Vanesa López-González, María José Sánchez-Soler, Lidya Rodriguez-Peña, Ana Teresa Serrano-Antón, Remedios Gil-Ferrer, Maria Del Carmen Martínez-Romero, Pablo Carbonell-Meseguer, Encarna Guillén-Navarro. High penetrance of acute intermittent porphyria in a Spanish founder mutation population and CYP2D6 genotype as a susceptibility factor. Orphanet journal of rare diseases. 2019 02; 14(1):59. doi: 10.1186/s13023-019-1031-7. [PMID: 30808393]
  • Eliane Sardh, Pauline Harper, Manisha Balwani, Penelope Stein, David Rees, D Montgomery Bissell, Robert Desnick, Charles Parker, John Phillips, Herbert L Bonkovsky, Daphne Vassiliou, Craig Penz, Amy Chan-Daniels, Qiuling He, William Querbes, Kevin Fitzgerald, Jae B Kim, Pushkal Garg, Akshay Vaishnaw, Amy R Simon, Karl E Anderson. Phase 1 Trial of an RNA Interference Therapy for Acute Intermittent Porphyria. The New England journal of medicine. 2019 02; 380(6):549-558. doi: 10.1056/nejmoa1807838. [PMID: 30726693]
  • Yi Ren, Lin-Xin Xu, Yun-Feng Liu, Chen-Yu Xiang, Fei Gao, Yan Wang, Tao Bai, Jian-Hong Yin, Yang-Lu Zhao, Jing Yang. A novel 55-basepair deletion of hydroxymethylbilane synthase gene found in a Chinese patient with acute intermittent porphyria and her family: A case report. Medicine. 2018 Sep; 97(37):e12295. doi: 10.1097/md.0000000000012295. [PMID: 30212967]
  • Silvina Fernanda Ruspini, Johanna Romina Zuccoli, Jimena Verónica Lavandera, Marìa Del Carmen Martínez, Leda María Oliveri, Esther Noemí Gerez, Alcira María Del Carmen Batlle, Ana María Buzaleh. Effects of volatile anaesthetics on heme metabolism in a murine genetic model of Acute Intermittent Porphyria. A comparative study with other porphyrinogenic drugs. Biochimica et biophysica acta. General subjects. 2018 06; 1862(6):1296-1305. doi: 10.1016/j.bbagen.2018.02.013. [PMID: 29476795]
  • Alexander Lang, Christian Heckl, Michael Vogeser, Thomas Stauch, Christian Homann, Georg Hennig, Ronald Sroka, Herbert Stepp. Rapid spectrophotometric quantification of urinary porphyrins and porphobilinogen as screening tool for attacks of acute porphyria. Journal of biomedical optics. 2018 05; 23(5):1-12. doi: 10.1117/1.jbo.23.5.055006. [PMID: 29855177]
  • Jon-Duri Senn, Lars C Huber, Jasmin Barman-Aksözen, Elisabeth Minder. [CME: Acute Intermittent Porphyria: When to Think of It? What Must be Checked? How to Treat?]. Praxis. 2018 Jan; 107(3):117-124. doi: 10.1024/1661-8157/a002887. [PMID: 29382268]
  • Jacqueline Woolf, Joanne T Marsden, Timothy Degg, Sharon Whatley, Paul Reed, Nadia Brazil, M Felicity Stewart, Michael Badminton. Best practice guidelines on first-line laboratory testing for porphyria. Annals of clinical biochemistry. 2017 Mar; 54(2):188-198. doi: 10.1177/0004563216667965. [PMID: 27555665]
  • Andreas Paulus, Marco Maenen, Natascha Drude, Emmani B M Nascimento, Wouter D van Marken Lichtenbelt, Felix M Mottaghy, Matthias Bauwens. Synthesis, radiosynthesis and in vitro evaluation of 18F-Bodipy-C16/triglyceride as a dual modal imaging agent for brown adipose tissue. PloS one. 2017; 12(8):e0182297. doi: 10.1371/journal.pone.0182297. [PMID: 28817670]
  • Manisha Balwani, Preeti Singh, Anju Seth, Ekta Malik Debnath, Hetanshi Naik, Dana Doheny, Brenden Chen, Makiko Yasuda, Robert J Desnick. Acute Intermittent Porphyria in children: A case report and review of the literature. Molecular genetics and metabolism. 2016 12; 119(4):295-299. doi: 10.1016/j.ymgme.2016.10.005. [PMID: 27769855]
  • Sumant Arora, Steven Young, Sudha Kodali, Ashwani K Singal. Hepatic porphyria: A narrative review. Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology. 2016 Nov; 35(6):405-418. doi: 10.1007/s12664-016-0698-0. [PMID: 27796941]
  • Nicholette M Oosthuizen, Janine Olivier, Janine Martins, Clara Schutte, Tahir S Pillay. Red-Brown Urine in a Patient with Chronic HIV Infection and Quadriparesis. Clinical chemistry. 2016 09; 62(9):1181-4. doi: 10.1373/clinchem.2015.250746. [PMID: 27573452]
  • Rosanna Grimes, Yvonne Gilleece, Timothy Appleby, Sarah Stockwell, Nicolas Pinto-Sander, Tatyana Sahabandu, Penelope Stein, Daniel Bradshaw. A case of hereditary coproporphyria precipitated by efavirenz. AIDS (London, England). 2016 08; 30(13):2142-3. doi: 10.1097/qad.0000000000001173. [PMID: 27465283]
  • Rushad Patell, Rupal Dosi, Harsh Joshi, Purav Shah, Rishi Tripathi. Challenges in the successful management of a case of acute intermittent porphyria in India. Tropical doctor. 2016 Jul; 46(3):150-3. doi: 10.1177/0049475515610939. [PMID: 26472549]
  • Michael Johns, Sakineh Esmaeili Mohsen Abadi, Nehal Malik, Joshua Lee, William L Neumann, Smita Rausaria, Maryam Imani-Nejad, Timothy McPherson, Joseph Schober, Guim Kwon. Oral administration of SR-110, a peroxynitrite decomposing catalyst, enhances glucose homeostasis, insulin signaling, and islet architecture in B6D2F1 mice fed a high fat diet. Archives of biochemistry and biophysics. 2016 04; 596(?):126-37. doi: 10.1016/j.abb.2016.03.002. [PMID: 26970045]
  • Tobias Braun, Christian Kleusch, Elena Naumovska, Rudolf Merkel, Agnes Csiszár. A bioanalytical assay to distinguish cellular uptake routes for liposomes. Cytometry. Part A : the journal of the International Society for Analytical Cytology. 2016 Mar; 89(3):301-8. doi: 10.1002/cyto.a.22792. [PMID: 26551759]
  • Yu S Marfin, E L Aleksakhina, D A Merkushev, E V Rumyantsev, I K Tomilova. Interaction of BODIPY Dyes with the Blood Plasma Proteins. Journal of fluorescence. 2016 Jan; 26(1):255-61. doi: 10.1007/s10895-015-1707-x. [PMID: 26520852]
  • Urszula Jarocka, Róża Sawicka, Anna Stachyra, Anna Góra-Sochacka, Agnieszka Sirko, Włodzimierz Zagórski-Ostoja, Violetta Sączyńska, Anna Porębska, Wim Dehaen, Jerzy Radecki, Hanna Radecka. A biosensor based on electroactive dipyrromethene-Cu(II) layer deposited onto gold electrodes for the detection of antibodies against avian influenza virus type H5N1 in hen sera. Analytical and bioanalytical chemistry. 2015 Oct; 407(25):7807-14. doi: 10.1007/s00216-015-8949-y. [PMID: 26297459]
  • Nicolas Pallet, Iadh Mami, Caroline Schmitt, Zoubida Karim, Arnaud François, Marion Rabant, Dominique Nochy, Laurent Gouya, Jean-Charles Deybach, Yichum Xu-Dubois, Eric Thervet, Hervé Puy, Alexandre Karras. High prevalence of and potential mechanisms for chronic kidney disease in patients with acute intermittent porphyria. Kidney international. 2015 Aug; 88(2):386-95. doi: 10.1038/ki.2015.97. [PMID: 25830761]
  • Josep Marcos, Maria Ibañez, Rosa Ventura, Jordi Segura, Jordi To-Figueras, Oscar J Pozo. Mass spectrometric characterisation of a condensation product between porphobilinogen and indolyl-3-acryloylglycine in urine of patients with acute intermittent porphyria. Journal of mass spectrometry : JMS. 2015 Jul; 50(7):929-37. doi: 10.1002/jms.3603. [PMID: 26349648]
  • Feiyi Wang, Ying Zhu, Li Zhou, Liang Pan, Zhifen Cui, Qiang Fei, Sihang Luo, Dun Pan, Qing Huang, Rui Wang, Chunchang Zhao, He Tian, Chunhai Fan. Fluorescent In Situ Targeting Probes for Rapid Imaging of Ovarian-Cancer-Specific γ-Glutamyltranspeptidase. Angewandte Chemie (International ed. in English). 2015 Jun; 54(25):7349-53. doi: 10.1002/anie.201502899. [PMID: 25940513]
  • Makiko Yasuda, Angelika L Erwin, Lawrence U Liu, Manisha Balwani, Brenden Chen, Senkottuvelan Kadirvel, Lin Gan, M Isabel Fiel, Ronald E Gordon, Chunli Yu, Sonia Clavero, Antonios Arvelakis, Hetanshi Naik, L David Martin, John D Phillips, Karl E Anderson, Vaithamanithi M Sadagoparamanujam, Sander S Florman, Robert J Desnick. Liver Transplantation for Acute Intermittent Porphyria: Biochemical and Pathologic Studies of the Explanted Liver. Molecular medicine (Cambridge, Mass.). 2015 Jun; 21(?):487-95. doi: 10.2119/molmed.2015.00099. [PMID: 26062020]
  • Albert W Girotti, Tamas Kriska. Binding and cytotoxic trafficking of cholesterol hydroperoxides by sterol carrier protein-2. Methods in molecular biology (Clifton, N.J.). 2015; 1208(?):421-35. doi: 10.1007/978-1-4939-1441-8_30. [PMID: 25323524]
  • Imran Ahmed, Ejaz Ahmed, Badshah Hussain Zaidi, Jawad Khaliq Ansari, Shazia Nisar. A YOUNG GIRL WITH FITS. Journal of Ayub Medical College, Abbottabad : JAMC. 2015 Jan; 27(1):232-3. doi: NULL. [PMID: 26182784]
  • Nicola Mumoli, Josè Vitale, Silvia Sabatini, Massimo Cocciolo, Lisa Gambaccini, Lorenzo Masi, Marco Cei. Prevalence and clinical features of acute intermittent porphyria: a retrospective analysis. Journal of the American Geriatrics Society. 2014 Sep; 62(9):1813-4. doi: 10.1111/jgs.12991. [PMID: 25243696]
  • H S Füessl. [Dark red urine]. MMW Fortschritte der Medizin. 2014 Apr; 156(7):33. doi: NULL. [PMID: 24956650]
  • Nicola Mumoli, Josè Vitale, Marco Cei. Images in emergency medicine. Acute intermittent porphyria. Annals of emergency medicine. 2014 Feb; 63(2):267, 273. doi: 10.1016/j.annemergmed.2013.06.013. [PMID: 24438652]
  • Joanne T Marsden, David C Rees. Urinary excretion of porphyrins, porphobilinogen and δ-aminolaevulinic acid following an attack of acute intermittent porphyria. Journal of clinical pathology. 2014 Jan; 67(1):60-5. doi: 10.1136/jclinpath-2012-201367. [PMID: 23908454]
  • Zhaohui Yin, Staffan Wahlin, Ewa C S Ellis, Pauline Harper, Bo-Göran Ericzon, Greg Nowak. Hepatocyte transplantation ameliorates the metabolic abnormality in a mouse model of acute intermittent porphyria. Cell transplantation. 2014; 23(9):1153-62. doi: 10.3727/096368913x666980. [PMID: 23582197]
  • Kristiina Kanerva, Riikka-Liisa Uronen, Tomas Blom, Shiqian Li, Robert Bittman, Pekka Lappalainen, Johan Peränen, Graça Raposo, Elina Ikonen. LDL cholesterol recycles to the plasma membrane via a Rab8a-Myosin5b-actin-dependent membrane transport route. Developmental cell. 2013 Nov; 27(3):249-62. doi: 10.1016/j.devcel.2013.09.016. [PMID: 24209575]
  • Conghua Mou, Xiaoran Hao, Zhixiang Xu, Xuguang Qiao. Effect of porphobilinogen on the formation of garlic green pigments. Journal of the science of food and agriculture. 2013 Aug; 93(10):2454-7. doi: 10.1002/jsfa.6056. [PMID: 23436238]
  • R V Dosi, A P Ambaliya, R D Patell, N N Sonune. Challenges in the diagnosis and treatment of a case of acute intermittent porphyria in India. Journal of postgraduate medicine. 2013 Jul; 59(3):241-2. doi: 10.4103/0022-3859.118056. [PMID: 24029211]
  • Sebastian Larion, Frederick R Caballes, Sun-Il Hwang, Jin-Gyun Lee, Whitney E Rossman, Judy Parsons, Nury Steuerwald, Ting Li, Vinaya Maddukuri, Gale Groseclose, Carla V Finkielstein, Herbert L Bonkovsky. Circadian rhythms in acute intermittent porphyria--a pilot study. European journal of clinical investigation. 2013 Jul; 43(7):727-39. doi: 10.1111/eci.12102. [PMID: 23650938]
  • Christopher M Benton, Lewis Couchman, Joanne T Marsden, David C Rees, Caje Moniz, Chang Kee Lim. Direct and simultaneous quantitation of 5-aminolaevulinic acid and porphobilinogen in human serum or plasma by hydrophilic interaction liquid chromatography-atmospheric pressure chemical ionization/tandem mass spectrometry. Biomedical chromatography : BMC. 2013 Feb; 27(2):267-72. doi: 10.1002/bmc.2843. [PMID: 23180457]
  • Víctor Quesada, Raquel Sarmiento-Mañús, Rebeca González-Bayón, Andrea Hricová, María Rosa Ponce, José Luis Micol. PORPHOBILINOGEN DEAMINASE deficiency alters vegetative and reproductive development and causes lesions in Arabidopsis. PloS one. 2013; 8(1):e53378. doi: 10.1371/journal.pone.0053378. [PMID: 23308205]
  • A Roberts, R Gill, R J Hussey, H Mikolajek, P T Erskine, J B Cooper, S P Wood, E J T Chrystal, P M Shoolingin-Jordan. Crystallization and preliminary X-ray characterization of the tetrapyrrole-biosynthetic enzyme porphobilinogen deaminase from Arabidopsis thaliana. Acta crystallographica. Section F, Structural biology and crystallization communications. 2012 Dec; 68(Pt 12):1491-3. doi: 10.1107/s1744309112042212. [PMID: 23192030]
  • Jonathan S Bogan, Yingke Xu, Mingming Hao. Cholesterol accumulation increases insulin granule size and impairs membrane trafficking. Traffic (Copenhagen, Denmark). 2012 Nov; 13(11):1466-80. doi: 10.1111/j.1600-0854.2012.01407.x. [PMID: 22889194]
  • Vijay K Dalal, Baishnab C Tripathy. Modulation of chlorophyll biosynthesis by water stress in rice seedlings during chloroplast biogenesis. Plant, cell & environment. 2012 Sep; 35(9):1685-703. doi: 10.1111/j.1365-3040.2012.02520.x. [PMID: 22494411]
  • Lucélia Moraes-Silva, Tania M Bueno, Carina Franciscato, Rafael P Ineu, Maria Ester Pereira. Effectiveness of copper chloride in protecting against alterations induced by mercury chloride in newborn rats. Journal of biochemical and molecular toxicology. 2012 Sep; 26(9):354-9. doi: 10.1002/jbt.21429. [PMID: 22811316]
  • Christopher M Benton, Lewis Couchman, Joanne T Marsden, David C Rees, Caje Moniz, Chang Kee Lim. Direct and simultaneous determination of 5-aminolaevulinic acid and porphobilinogen in urine by hydrophilic interaction liquid chromatography-electrospray ionisation/tandem mass spectrometry. Biomedical chromatography : BMC. 2012 Aug; 26(8):1033-40. doi: 10.1002/bmc.2770. [PMID: 22740490]
  • Elizabeth Anyaegbu, Michael Goodman, Sun-Young Ahn, Mathula Thangarajh, Michael Wong, Marwan Shinawi. Acute intermittent porphyria: a diagnostic challenge. Journal of child neurology. 2012 Jul; 27(7):917-21. doi: 10.1177/0883073811427603. [PMID: 22190498]
  • P E Stein, M N Badminton, J H Barth, D C Rees, R Sarkany, M F Stewart, T M Cox. Acute intermittent porphyria: fatal complications of treatment. Clinical medicine (London, England). 2012 Jun; 12(3):293-4. doi: 10.7861/clinmedicine.12-3-293. [PMID: 22783787]
  • Andrei M Beliaev, Antonio Foliaki, Leah Ha, D Ross Boswell. Acute non-specific abdominal pain with port-wine urine. ANZ journal of surgery. 2012 Jun; 82(6):478-9. doi: 10.1111/j.1445-2197.2012.06089.x. [PMID: 22672404]
  • Marta Moya, Marta Benet, Carla Guzmán, Laia Tolosa, Carmelo García-Monzón, Eugenia Pareja, José Vicente Castell, Ramiro Jover. Foxa1 reduces lipid accumulation in human hepatocytes and is down-regulated in nonalcoholic fatty liver. PloS one. 2012; 7(1):e30014. doi: 10.1371/journal.pone.0030014. [PMID: 22238690]
  • Carmen Unzu, Ana Sampedro, Eliane Sardh, Itsaso Mauleón, Rafael Enríquez de Salamanca, Jesús Prieto, Eduardo Salido, Pauline Harper, Antonio Fontanellas. Renal failure affects the enzymatic activities of the three first steps in hepatic heme biosynthesis in the acute intermittent porphyria mouse. PloS one. 2012; 7(3):e32978. doi: 10.1371/journal.pone.0032978. [PMID: 22412963]
  • Inimioara Mihaela Cojocaru, Violeta Sapira, Gabriela Socoliuc, Cristina Hertea, M Balea, Carmen Ursache, M Cojocaru. Acute intermittent porphyria--diagnostic and treatment traps. Romanian journal of internal medicine = Revue roumaine de medecine interne. 2012 Jan; 50(1):33-41. doi: NULL. [PMID: 22788092]
  • Longhou Fang, Simone R Green, Ji Sun Baek, Sang-Hak Lee, Felix Ellett, Elena Deer, Graham J Lieschke, Joseph L Witztum, Sotirios Tsimikas, Yury I Miller. In vivo visualization and attenuation of oxidized lipid accumulation in hypercholesterolemic zebrafish. The Journal of clinical investigation. 2011 Dec; 121(12):4861-9. doi: 10.1172/jci57755. [PMID: 22105168]
  • Nilima N Dongre, Adinath N Suryakar, Arun J Patil, Jeevan G Ambekar, Dileep B Rathi. Biochemical effects of lead exposure on systolic & diastolic blood pressure, heme biosynthesis and hematological parameters in automobile workers of north karnataka (India). Indian journal of clinical biochemistry : IJCB. 2011 Oct; 26(4):400-6. doi: 10.1007/s12291-011-0159-6. [PMID: 23024478]
  • Michael Vogeser, Thomas Stauch. Evaluation of a commercially available rapid urinary porphobilinogen test. Clinical chemistry and laboratory medicine. 2011 Sep; 49(9):1491-4. doi: 10.1515/cclm.2011.605. [PMID: 21627493]
  • Jinglan Zhang, Makiko Yasuda, Robert J Desnick, Manisha Balwani, David Bishop, Chunli Yu. A LC-MS/MS method for the specific, sensitive, and simultaneous quantification of 5-aminolevulinic acid and porphobilinogen. Journal of chromatography. B, Analytical technologies in the biomedical and life sciences. 2011 Aug; 879(24):2389-96. doi: 10.1016/j.jchromb.2011.06.034. [PMID: 21783436]
  • Zoran Gucev, Nevenka Slavevska, Velibor Tasic, Nevenka Laban, Nada Pop-Jordanova, Dragan Danilovski, Jacqueline Woolf, Duncan Cole. Congenital erythropoietic porphyria with two mutations of the uroporphyrinogen III synthase gene (Cys73Arg, Thr228Met). Indian journal of human genetics. 2011 May; 17(2):104-7. doi: 10.4103/0971-6866.86199. [PMID: 22090724]
  • Ellen Ma, Victoria Mar, George Varigos, Amanda Nicoll, Gayle Ross. Haem arginate as effective maintenance therapy for hereditary coproporphyria. The Australasian journal of dermatology. 2011 May; 52(2):135-8. doi: 10.1111/j.1440-0960.2011.00747.x. [PMID: 21605099]
  • Roman Sobotka, Martin Tichy, Annegret Wilde, C Neil Hunter. Functional assignments for the carboxyl-terminal domains of the ferrochelatase from Synechocystis PCC 6803: the CAB domain plays a regulatory role, and region II is essential for catalysis. Plant physiology. 2011 Apr; 155(4):1735-47. doi: 10.1104/pp.110.167528. [PMID: 21081693]
  • Jian Wang, Kostas Pantopoulos. Regulation of cellular iron metabolism. The Biochemical journal. 2011 Mar; 434(3):365-81. doi: 10.1042/bj20101825. [PMID: 21348856]
  • Ludovic D'auria, Patrick Van der Smissen, Frédéric Bruyneel, Pierre J Courtoy, Donatienne Tyteca. Segregation of fluorescent membrane lipids into distinct micrometric domains: evidence for phase compartmentation of natural lipids?. PloS one. 2011 Feb; 6(2):e17021. doi: 10.1371/journal.pone.0017021. [PMID: 21386970]
  • Celine Vanhee, Grzegorz Zapotoczny, Danièle Masquelier, Michel Ghislain, Henri Batoko. The Arabidopsis multistress regulator TSPO is a heme binding membrane protein and a potential scavenger of porphyrins via an autophagy-dependent degradation mechanism. The Plant cell. 2011 Feb; 23(2):785-805. doi: 10.1105/tpc.110.081570. [PMID: 21317376]
  • Ching-Wan Lam, Kin-Chong Lau, Chloe Miu Mak, Man-Wo Tsang, Yan-Wo Chan. Circulating fluorocytes at the first attack of acute intermittent porphyria: a missing link in the pathogenesis. Clinica chimica acta; international journal of clinical chemistry. 2011 Jan; 412(1-2):208-12. doi: 10.1016/j.cca.2010.09.005. [PMID: 20850424]
  • Miroslav Mydlík, Katarína Derzsiová. Kidney damage in acute intermittent porphyria. Przeglad lekarski. 2011; 68(9):610-3. doi: NULL. [PMID: 22335011]
  • Sankha Koley, Vikrant Saoji. Congenital erythropoietic porphyria: two case reports. Indian journal of dermatology. 2011 Jan; 56(1):94-7. doi: 10.4103/0019-5154.77565. [PMID: 21572804]
  • Thomas Bollinger, Anton Leutz, Alexei Leliavski, Ludmila Skrum, Judit Kovac, Luigi Bonacina, Christian Benedict, Tanja Lange, Jürgen Westermann, Henrik Oster, Werner Solbach. Circadian clocks in mouse and human CD4+ T cells. PloS one. 2011; 6(12):e29801. doi: 10.1371/journal.pone.0029801. [PMID: 22216357]
  • Tomoko Masuda, Rie Ota, Takao Ando, Naoto Maeda, Yutaka Horie, Toshiro Yoshimura, Masakatsu Motomura, Atsushi Kawakami. False-positive accumulation of metaiodobenzylguanidine in a case with acute intermittent porphyria. Internal medicine (Tokyo, Japan). 2011; 50(9):1029-32. doi: 10.2169/internalmedicine.50.5096. [PMID: 21532227]
  • Harvey C Gonick. Lead-binding proteins: a review. Journal of toxicology. 2011; 2011(?):686050. doi: 10.1155/2011/686050. [PMID: 21941540]
  • Hung-Chou Kuo, Chin-Chang Huang, Chun-Che Chu, Ming-Jen Lee, Wen-Li Chuang, Chi-Lin Wu, Tony Wu, Hsiao-Chen Ning, Chih-Yang Liu. Neurological complications of acute intermittent porphyria. European neurology. 2011; 66(5):247-52. doi: 10.1159/000330683. [PMID: 21986212]
  • Olaf Czarnecki, Enrico Peter, Bernhard Grimm. Methods for analysis of photosynthetic pigments and steady-state levels of intermediates of tetrapyrrole biosynthesis. Methods in molecular biology (Clifton, N.J.). 2011; 775(?):357-85. doi: 10.1007/978-1-61779-237-3_20. [PMID: 21863454]
  • Pierre Bouizegarene, Hervé Puy, Vasco Pereira da Silva, Jean-Charles Deybach. [Porphyria. What to think in the absence of a cause of abdominal pain]. La Revue du praticien. 2010 Dec; 60(10):1341-4. doi: NULL. [PMID: 21425523]
  • Irene Paradisi, Sergio Arias. Marked geographic aggregation of acute intermittent porphyria families carrying mutation Q180X in Venezuelan populations, with description of further mutations. Journal of inherited metabolic disease. 2010 Dec; 33 Suppl 3(?):S455-63. doi: 10.1007/s10545-010-9228-x. [PMID: 20978940]
  • Frederick A Heberle, Jing Wu, Shih Lin Goh, Robin S Petruzielo, Gerald W Feigenson. Comparison of three ternary lipid bilayer mixtures: FRET and ESR reveal nanodomains. Biophysical journal. 2010 Nov; 99(10):3309-18. doi: 10.1016/j.bpj.2010.09.064. [PMID: 21081079]
  • R T Sitaram, S Degerman, B Ljungberg, E Andersson, Y Oji, H Sugiyama, G Roos, A Li. Wilms' tumour 1 can suppress hTERT gene expression and telomerase activity in clear cell renal cell carcinoma via multiple pathways. British journal of cancer. 2010 Oct; 103(8):1255-62. doi: 10.1038/sj.bjc.6605878. [PMID: 20842112]
  • Maria Domenica Cappellini, Valentina Brancaleoni, Giovanna Graziadei, Dario Tavazzi, Elena Di Pierro. Porphyrias at a glance: diagnosis and treatment. Internal and emergency medicine. 2010 Oct; 5 Suppl 1(?):S73-80. doi: 10.1007/s11739-010-0449-7. [PMID: 20865478]
  • Premysl Pejchar, Martin Potocký, Zuzana Novotná, Stepánka Veselková, Daniela Kocourková, Olga Valentová, Katerina Schwarzerová, Jan Martinec. Aluminium ions inhibit the formation of diacylglycerol generated by phosphatidylcholine-hydrolysing phospholipase C in tobacco cells. The New phytologist. 2010 Oct; 188(1):150-60. doi: 10.1111/j.1469-8137.2010.03349.x. [PMID: 20629955]
  • Philip R A Webber, Aasne K Aarsand, Arne K Sandvik, Øyvind Skadberg, Morten Lindberg, Sverre Sandberg. Acute porphyrias may be overlooked in patients taking methenamine hippurate. Clinical chemistry and laboratory medicine. 2010 Sep; 48(9):1355-7. doi: 10.1515/cclm.2010.253. [PMID: 20557279]
  • Sonia Clavero, David F Bishop, Urs Giger, Mark E Haskins, Robert J Desnick. Feline congenital erythropoietic porphyria: two homozygous UROS missense mutations cause the enzyme deficiency and porphyrin accumulation. Molecular medicine (Cambridge, Mass.). 2010 Sep; 16(9-10):381-8. doi: 10.2119/molmed.2010.00038. [PMID: 20485863]
  • Matej Vesteg, Rostislav Vacula, Jürgen M Steiner, Bianka Mateásiková, Wolfgang Löffelhardt, Brona Brejová, Juraj Krajcovic. A possible role for short introns in the acquisition of stroma-targeting peptides in the flagellate Euglena gracilis. DNA research : an international journal for rapid publication of reports on genes and genomes. 2010 Aug; 17(4):223-31. doi: 10.1093/dnares/dsq015. [PMID: 20587589]
  • Biserka Bakrac, Ales Kladnik, Peter Macek, Gavin McHaffie, Andreas Werner, Jeremy H Lakey, Gregor Anderluh. A toxin-based probe reveals cytoplasmic exposure of Golgi sphingomyelin. The Journal of biological chemistry. 2010 Jul; 285(29):22186-95. doi: 10.1074/jbc.m110.105122. [PMID: 20463009]
  • Dhanasekaran Shanmugam, Bo Wu, Ursula Ramirez, Eileen K Jaffe, David S Roos. Plastid-associated porphobilinogen synthase from Toxoplasma gondii: kinetic and structural properties validate therapeutic potential. The Journal of biological chemistry. 2010 Jul; 285(29):22122-31. doi: 10.1074/jbc.m110.107243. [PMID: 20442414]
  • Prakash G Chandak, Branislav Radovic, Elma Aflaki, Dagmar Kolb, Marlene Buchebner, Eleonore Fröhlich, Christoph Magnes, Frank Sinner, Guenter Haemmerle, Rudolf Zechner, Ira Tabas, Sanja Levak-Frank, Dagmar Kratky. Efficient phagocytosis requires triacylglycerol hydrolysis by adipose triglyceride lipase. The Journal of biological chemistry. 2010 Jun; 285(26):20192-201. doi: 10.1074/jbc.m110.107854. [PMID: 20424161]
  • Hamidreza Shirvani-Mahdavi, Shima Fardad, Ezeddin Mohajerani, Shin-Tson Wu. High efficiency cholesteric liquid crystal lasers with an external stable resonator. Optics express. 2010 Jun; 18(13):13593-9. doi: 10.1364/oe.18.013593. [PMID: 20588492]
  • Jens Bedke, Bernhard Hemmerlein, Christina Perske, Andreas Gross, Markus Heuser. Tumor-associated macrophages in clear cell renal cell carcinoma express both gastrin-releasing peptide and its receptor: a possible modulatory role of immune effectors cells. World journal of urology. 2010 Jun; 28(3):335-41. doi: 10.1007/s00345-009-0492-z. [PMID: 20012906]
  • Staffan Wahlin, Pauline Harper, Eliane Sardh, Christer Andersson, Dan Eh Andersson, Bo-Göran Ericzon. Combined liver and kidney transplantation in acute intermittent porphyria. Transplant international : official journal of the European Society for Organ Transplantation. 2010 Jun; 23(6):e18-21. doi: 10.1111/j.1432-2277.2009.01035.x. [PMID: 20028496]