Homocitrulline (BioDeep_00000003674)

 

Secondary id: BioDeep_00000402859, BioDeep_00000405333

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite natural product


代谢物信息卡片


(2S)-2-amino-6-(carbamoylamino)hexanoic acid

化学式: C7H15N3O3 (189.1113)
中文名称: L-高瓜氨酸, 高瓜氨酸, l-高瓜氨酸
谱图信息: 最多检出来源 Homo sapiens(blood) 23.1%

Reviewed

Last reviewed on 2024-06-29.

Cite this Page

Homocitrulline. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/homocitrulline (retrieved 2024-12-24) (BioDeep RN: BioDeep_00000003674). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: C(CCNC(=O)N)CC(C(=O)O)N
InChI: InChI=1S/C7H15N3O3/c8-5(6(11)12)3-1-2-4-10-7(9)13/h5H,1-4,8H2,(H,11,12)(H3,9,10,13)/t5-/m0/s1

描述信息

Homocitrulline is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders (OMIM 238970). The accumulation of carbamylphosphate due to depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of homocitrulline and homoarginine in some cases (PMID 2474087). Homocitrulline has been identified in the human placenta (PMID: 32033212).
Homocitrulline is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders (OMIM 238970). The accumulation of carbamylphosphate due to depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of homocitrulline and homoarginine in some cases (PMID 2474087). [HMDB]
L-Homocitrulline is metabolized to homoarginine through homoargininosuccinate via the urea cycle pathway and its metabolic abnormality could lead to Lysinuric Protein Intolerance (LPI).
L-Homocitrulline is metabolized to homoarginine through homoargininosuccinate via the urea cycle pathway and its metabolic abnormality could lead to Lysinuric Protein Intolerance (LPI).

同义名列表

18 个代谢物同义名

(2S)-2-amino-6-(carbamoylamino)hexanoic acid; (S)-2-Amino-6-ureidohexanoic acid; N-ε-Carbamyl-L-lysine; 2-Amino-6-ureidohexanoic acid; N(6)-(Aminocarbonyl)-L-lysine; N6-(Aminocarbonyl)-L-lysine; N-epsilon-Carbamyl-L-lysine; N-Ε-carbamyl-L-lysine; N-e-Carbamyl-L-lysine; N6-Carbamoyl-L-lysine; N6-Carbamoyl-lysine; Ureidocaproic acid; Homo-L-citrulline; L-Homocitrulline; Homocitrulline; Homocitrulline; L-Homocitrulline; L-Homocitrulline



数据库引用编号

19 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

0 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(0)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(0)

PharmGKB(0)

10 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。

亚细胞结构定位 关联基因列表
Cytoplasm 8 ALB, ARG1, CAT, ENO1, PADI2, PADI3, PADI4, VIM
Endoplasmic reticulum membrane 1 CD4
Mitochondrion membrane 2 ORC1, SLC25A15
Nucleus 8 ALB, ARG1, ENO1, MPO, ORC1, PADI2, PADI3, PADI4
cytosol 10 ALB, ARG1, CAT, CBL, ENO1, ORC1, PADI2, PADI3, PADI4, VIM
phagocytic vesicle 1 VIM
centrosome 1 ALB
nucleoplasm 5 ATP2B1, MPO, ORC1, PADI3, PADI4
Cell membrane 4 ATP2B1, CD4, ENO1, VIM
Cytoplasmic granule 2 ARG1, PADI4
Multi-pass membrane protein 5 ATP2B1, ORC1, SLC25A15, SLC7A7, SPG7
Synapse 1 ATP2B1
cell cortex 1 ENO1
cell surface 1 ENO1
glutamatergic synapse 1 ATP2B1
Golgi apparatus 2 ALB, CBL
Golgi membrane 1 INS
growth cone 1 CBL
mitochondrial inner membrane 4 ORC1, OTC, SLC25A15, SPG7
presynaptic membrane 1 ATP2B1
Lysosome 1 MPO
plasma membrane 6 ATP2B1, CBL, CD4, ENO1, SLC7A7, VIM
synaptic vesicle membrane 1 ATP2B1
Membrane 7 ATP2B1, CAT, ENO1, ORC1, SLC25A15, SLC7A7, SPG7
axon 1 VIM
basolateral plasma membrane 2 ATP2B1, SLC7A7
extracellular exosome 7 ALB, ATP2B1, CAT, ENO1, MPO, PADI2, VIM
endoplasmic reticulum 1 ALB
extracellular space 7 ALB, ARG1, CRP, CXCL8, ENO1, INS, MPO
perinuclear region of cytoplasm 1 CBL
mitochondrion 5 CAT, ORC1, OTC, SLC25A15, SPG7
protein-containing complex 3 ALB, CAT, PADI4
intracellular membrane-bounded organelle 4 ATP2B1, CAT, MPO, PADI3
Single-pass type I membrane protein 1 CD4
Secreted 4 ALB, CRP, CXCL8, INS
extracellular region 8 ALB, ARG1, CAT, CRP, CXCL8, INS, MPO, PADI2
Mitochondrion matrix 1 OTC
mitochondrial matrix 2 CAT, OTC
anchoring junction 1 ALB
Cell projection, cilium 1 CBL
Cytoplasmic vesicle, secretory vesicle, synaptic vesicle membrane 1 ATP2B1
external side of plasma membrane 1 CD4
axon cytoplasm 1 SPG7
Early endosome 1 CD4
Mitochondrion inner membrane 3 ORC1, SLC25A15, SPG7
Membrane raft 2 CBL, CD4
Cytoplasm, cytoskeleton 1 VIM
focal adhesion 3 CAT, CBL, VIM
flotillin complex 1 CBL
Peroxisome 2 CAT, VIM
Peroxisome matrix 1 CAT
peroxisomal matrix 1 CAT
peroxisomal membrane 1 CAT
secretory granule 1 MPO
intermediate filament 1 VIM
lateral plasma membrane 1 ATP2B1
nuclear outer membrane 1 ENO1
neuron projection 1 VIM
ciliary basal body 1 ALB
cilium 1 CBL
cell leading edge 1 VIM
cell projection 1 ATP2B1
cytoskeleton 1 VIM
centriole 1 ALB
spindle pole 1 ALB
chromosome, telomeric region 1 ORC1
blood microparticle 1 ALB
Basolateral cell membrane 2 ATP2B1, SLC7A7
endosome lumen 1 INS
Cytoplasm, myofibril, sarcomere, M line 1 ENO1
M band 1 ENO1
microtubule organizing center 1 VIM
euchromatin 1 PADI2
Presynaptic cell membrane 1 ATP2B1
intermediate filament cytoskeleton 1 VIM
azurophil granule 1 MPO
ficolin-1-rich granule lumen 1 CAT
secretory granule lumen 2 CAT, INS
Golgi lumen 1 INS
endoplasmic reticulum lumen 3 ALB, CD4, INS
nuclear matrix 1 VIM
platelet alpha granule lumen 1 ALB
specific granule lumen 1 ARG1
transport vesicle 1 INS
azurophil granule lumen 3 ARG1, MPO, PADI2
Endoplasmic reticulum-Golgi intermediate compartment membrane 1 INS
immunological synapse 1 ATP2B1
Nucleus matrix 1 VIM
clathrin-coated endocytic vesicle membrane 1 CD4
phagocytic vesicle lumen 1 MPO
nuclear origin of replication recognition complex 1 ORC1
origin recognition complex 1 ORC1
T cell receptor complex 1 CD4
catalase complex 1 CAT
mitochondrial permeability transition pore complex 1 SPG7
phosphopyruvate hydratase complex 1 ENO1
photoreceptor ribbon synapse 1 ATP2B1
ciliary transition fiber 1 ALB
m-AAA complex 1 SPG7


文献列表

  • Svetlana Baskal, Alexander Bollenbach, Dimitrios Tsikas. GC-MS Discrimination of Citrulline from Ornithine and Homocitrulline from Lysine by Chemical Derivatization: Evidence of Formation of N5-Carboxy-ornithine and N6-Carboxy-lysine. Molecules (Basel, Switzerland). 2021 Apr; 26(8):. doi: 10.3390/molecules26082301. [PMID: 33921162]
  • Emanuele De Simone, Lucia Di Micco, Gaetano La Manna, Biagio Di Iorio. [Protein carbamylation: what it is and why it concerns nephrologists]. Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia. 2018 May; 35(3):. doi: NULL. [PMID: 29786184]
  • Stéphane Jaisson, Aurore Desmons, Manon Doué, Laëtitia Gorisse, Christine Pietrement, Philippe Gillery. Measurement of Homocitrulline, A Carbamylation-derived Product, in Serum and Tissues by LC-MS/MS. Current protocols in protein science. 2018 04; 92(1):e56. doi: 10.1002/cpps.56. [PMID: 30040196]
  • Michael S Lustgarten, Roger A Fielding. Metabolites related to renal function, immune activation, and carbamylation are associated with muscle composition in older adults. Experimental gerontology. 2017 12; 100(?):1-10. doi: 10.1016/j.exger.2017.10.003. [PMID: 29030163]
  • Morgan E Grams, Adrienne Tin, Casey M Rebholz, Tariq Shafi, Anna Köttgen, Ronald D Perrone, Mark J Sarnak, Lesley A Inker, Andrew S Levey, Josef Coresh. Metabolomic Alterations Associated with Cause of CKD. Clinical journal of the American Society of Nephrology : CJASN. 2017 Nov; 12(11):1787-1794. doi: 10.2215/cjn.02560317. [PMID: 28971980]
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  • Yuki Taga, Keisuke Tanaka, Chieko Hamada, Masashi Kusubata, Kiyoko Ogawa-Goto, Shunji Hattori. Hydroxyhomocitrulline Is a Collagen-Specific Carbamylation Mark that Affects Cross-link Formation. Cell chemical biology. 2017 Oct; 24(10):1276-1284.e3. doi: 10.1016/j.chembiol.2017.08.010. [PMID: 28943354]
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