Details of Metabolite

General Information of MET (ID: META00196)
Name Cystine
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(-)-Cystine', (R-(R*,r*))-3,3'-dithiobis, '2-Amino-3-(2-amino-2-carboxy-ethyl)disulfanyl-propanoate; (R,R)-3,3'-Dithiobis(2-aminopropanoic acid), (R-(R*,r*))-3,3'-dithiobis(2-aminopropanoic acid), 3,3'-Dithiobis-L-alanine, beta,Beta'-diamino-beta,beta'-dicarboxydiethyl disulfide, beta,Beta'-dithiodialanine, 'Bis(beta-amino-beta-carboxyethyl) disulfide; 2-Amino-3-(2-amino-2-carboxy-ethyl)disulfanyl-propanoic acid; 2-Amino-3-[(2-amino-2-carboxyethyl)dithio]propanoate; 2-Amino-3-[(2-amino-2-carboxyethyl)dithio]propanoic acid', 3,3'-Dithiobis, 3,3'-Dithiobis[2-amino-[R-(r*,r*)]-propanoate, 3,3'-Dithiobis[2-amino-[R-(r*,r*)]-propanoic acid, 3,3'-Dithiodialanine, b,B'-diamino-b,b'-dicarboxydiethyl disulfide, b,B'-dithiodialanine, beta,Beta'-dithiobisalanine, 'Bis(b-amino-beta-carboxyethyl) disulfide; Bis(b-amino-b-carboxyethyl) disulphide; Copper cystinate; Cysteine disulfide; Cystin; Cystine; Cystine acid', D(+)-3,3'-Dithiobis(2-aminopropanoate, D(+)-3,3'-Dithiobis(2-aminopropanoic acid, 'Dicysteine; Gelucystine; L-(-)-Cystine; L-Cysteine disulfide; L-Cystin', [R-(R*,r*)]-3,3'-dithiobis, 'L Cystine; L-Cystine; L-Dicysteine; L-a-Diamino-b-dithiolactate; L-a-Diamino-b-dithiolactic acid; L-alpha-Diamino-beta-dithiolactic acid; Oxidized L-cysteine', (R,R)-3,3'-Dithiobis(2-aminopropanoate), (R-(R*,r*))-3,3'-dithiobis(2-aminopropanoate), b,Beta'-diamino-b,beta'-dicarboxydiethyl disulfide, b,Beta'-diamino-b,beta'-dicarboxydiethyl disulphide, beta,Beta'-diamino-beta,beta'-dicarboxydiethyl disulphide, u\u0392,beta'-diamino-\u03b2,beta'-dicarboxydiethyl disulfide, u\u0392,beta'-diamino-\u03b2,beta'-dicarboxydiethyl disulphide, b,Beta'-dithiodialanine, u\u0392,beta'-dithiodialanine, 'Bis(b-amino-b-carboxyethyl) disulfide; e921
Source Endogenous;Escherichia Coli Metabolite;Yeast Metabolite;Food;Drug;Cosmetic;Food additives;Microbial
Structure Type   Amino acids, peptides, and analogues  (Click to Show/Hide the Complete Structure Type Hierarchy)
Organic acids and derivatives
Carboxylic acids and derivatives
Amino acids, peptides, and analogues
PubChem CID
67678
HMDB ID
HMDB0000192
Formula
C6H12N2O4S2
Structure
<iframe style="width: 300px; height: 300px;" frameborder="0" src="https://embed.molview.org/v1/?mode=balls&cid=67678"></iframe>
3D MOL 2D MOL
  Click to Show/Hide the Molecular/Functional Data (External Links/Property/Function) of This Metabolite
KEGG ID
C00491
DrugBank ID
DB00138
ChEBI ID
16283
FooDB ID
FDB012672
ChemSpider ID
60997
METLIN ID
5207
Physicochemical Properties Molecular Weight 240.3 Topological Polar Surface Area 177
XlogP -6.3 Complexity 192
Heavy Atom Count 14 Rotatable Bond Count 7
Hydrogen Bond Donor Count 4 Hydrogen Bond Acceptor Count 8
Function
Cystine is an oxidized dimeric form of cysteine. It is formed by linking two cysteine residues via a disulfide bond (Cys-S-S-Cys) between the -SH groups. Cystine is found in high concentrations in digestive enzymes and in the cells of the immune system, skeletal and connective tissues, skin, and hair. Hair and skin are 10-14% cystine. Cystine is the preferred form of cysteine for the synthesis of glutathione in cells involved in the immune system (e.g. macrophages and astrocytes). Lymphocytes and neurons prefer cysteine for glutathione production. Optimizing glutathione levels in macrophages and astrocytes with cystine allows these cells to provide cysteine to lymphocytes and neurons directly upon demand.
Regulatory Network
Full List of Protein(s) Regulating This Metabolite
      Amino acid/polyamine transporter (AAPT)
            Asc-type amino transporter 1 (SLC7A10) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Overexpression of SLC7A10
                      Induced Change Cystine concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that overexpression of SLC7A10 leads to the increase of cystine levels compared with control group.
            Cystine/glutamate transporter (SLC7A11) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [2]
                      Introduced Variation Overexpression of Slc7a11
                      Induced Change Cystine concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that overexpression of Slc7a11 leads to the increase of cystine levels compared with control group.
            Integral membrane E16 (SLC7A5) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [3]
                      Introduced Variation Overexperisson of SLC7A5
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that co-overexperisson of SLC7A5 and SLC7A8 leads to the decrease of cystine levels compared with control group.
            L-type amino acid transporter 2 (LAT2) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [3]
                      Introduced Variation Overexperisson of SLC7A8
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that co-overexperisson of SLC7A5 and SLC7A8 leads to the decrease of cystine levels compared with control group.
            Solute carrier family 7 member 9 (SLC7A9) Click to Show/Hide the Full List of Regulating Pair(s):   8 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation ( N227D) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation ( N227D) of SLC7A9 leads to the decrease of cystine levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (1105delA; W69stop) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation (1105delA; W69stop) of SLC7A9 leads to the decrease of cystine levels compared with control group.
               Regulating Pair (3) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (G195R) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation (G195R) of SLC7A9 leads to the decrease of cystine levels compared with control group.
               Regulating Pair (4) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (L223M) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation (L223M) of SLC7A9 leads to the decrease of cystine levels compared with control group.
               Regulating Pair (5) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (P482L) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation (P482L) of SLC7A9 leads to the decrease of cystine levels compared with control group.
               Regulating Pair (6) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (R333Q) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation (R333Q) of SLC7A9 leads to the decrease of cystine levels compared with control group.
               Regulating Pair (7) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (R333W) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation (R333W) of SLC7A9 leads to the decrease of cystine levels compared with control group.
               Regulating Pair (8) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (V142A; L223M) of SLC7A9
                      Induced Change Cystine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Nephropathic cystinosis [ICD-11: 5C60]
                      Details It is reported that mutation (V142A and L223M) of SLC7A9 leads to the decrease of cystine levels compared with control group.
      Oxidoreductases (EC 1)
            Glyoxylate reductase/hydroxypyruvate reductase (GRHPR) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [5]
                      Introduced Variation Mutation (patients: c.454dup (p.Thr152Asnfs*39)) of GRHPR
                      Induced Change Cystine concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Carbohydrate metabolism disorders [ICD-11: 5C51]
                      Details It is reported that mutation (patients with c.454dup (p.Thr152Asnfs*39)) of GRHPR leads to the increase of cystine levels compared with control group.
References
1 Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity. Neurosci Lett. 2000 Jun 30;287(3):231-5.
2 Cloning and expression of a plasma membrane cystine/glutamate exchange transporter composed of two distinct proteins. J Biol Chem. 1999 Apr 23;274(17):11455-8.
3 Identification of a membrane protein, LAT-2, that Co-expresses with 4F2 heavy chain, an L-type amino acid transport activity with broad specificity for small and large zwitterionic amino acids. J Biol Chem. 1999 Jul 9;274(28):19738-44.
4 A novel missense mutation of SLC7A9 frequent in Japanese cystinuria cases affecting the C-terminus of the transporter. Kidney Int. 2006 Apr;69(7):1198-206.
5 Severe child form of primary hyperoxaluria type 2 - a case report revealing consequence of GRHPR deficiency on metabolism. BMC Med Genet. 2017 May 31;18(1):59.

If you find any error in data or bug in web service, please kindly report it to Dr. Zhang and Dr. Mou.