Cystine

Identification

Summary

Cystine is an oxidated derivative of the amino acid cysteine found in various nutritional products, acne treatments, and creams to treat cervical injury or inflammation.

Generic Name
Cystine
DrugBank Accession Number
DB00138
Background

A covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine.

Type
Small Molecule
Groups
Approved, Nutraceutical
Structure
Weight
Average: 240.3
Monoisotopic: 240.023848262
Chemical Formula
C6H12N2O4S2
Synonyms
  • (R,R)-3,3'-dithiobis(2-aminopropanoic acid)
  • Cistina
  • Cystine
  • L-alpha-Diamino-beta-dithiolactic acid
  • L-Cystine
  • L-Dicysteine
  • β,β'-dithiodialanine

Pharmacology

Indication

It has been claimed that L-cysteine has anti-inflammatory properties, that it can protect against various toxins, and that it might be helpful in osteoarthritis and rheumatoid arthritis. More research will have to be done before L-cysteine can be indicated for any of these conditions. Research to date has mostly been in animal models.

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Associated Conditions
Indication TypeIndicationCombined Product DetailsApproval LevelAge GroupPatient CharacteristicsDose Form
Adjunct therapy in treatment ofAcne••• •••••••••
Used in combination to treatAlopeciaCombination Product in combination with: Pantothenic acid (DB01783)••• •••••••••••••••
Adjunct therapy in treatment ofAtrophic dermatitis••• •••••••••
Used in combination to treatHair damageCombination Product in combination with: Pantothenic acid (DB01783)••• •••••••••••••••
Used in combination to treatNail growth abnormalCombination Product in combination with: Pantothenic acid (DB01783)••• •••••••••••••••
Associated Therapies
Contraindications & Blackbox Warnings
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Pharmacodynamics

L-Cystine is a covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. Cystine is a chemical substance which naturally occurs as a deposit in the urine, and can form a calculus (hard mineral formation) when deposited in the kidney. The compound produced when two cysteine molecules linked by a disulfide (S-S) bond. Cystine is required for proper vitamin B6 utilization and is also helpful in the healing of burns and wounds, breaking down mucus deposits in illnesses such as bronchitis as well as cystic fibrosis. Cysteine also assists in the supply of insulin to the pancreas, which is needed for the assimilation of sugars and starches. It increases the level of glutathione in the lungs, liver, kidneys and bone marrow, and this may have an anti-aging effect on the body by reducing age-spots etc.

Mechanism of action

Certain conditions, e.g. an acetaminophen overdose, deplete hepatic glutathione and subject the tissues to oxidative stress resulting in loss of cellular integrity. L-Cystine serves as a major precursor for synthesis of glutathione.

TargetActionsOrganism
UCystine/glutamate transporterNot AvailableHumans
UCystinosinNot AvailableHumans
Absorption

Not Available

Volume of distribution

Not Available

Protein binding

Not Available

Metabolism
Not Available
Route of elimination

Not Available

Half-life

Not Available

Clearance

Not Available

Adverse Effects
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Toxicity

With typical doses of 1 to 1.5 grams daily, the most commonly reported side effects have been gastrointestinal, such as nausea. There are rare reports of cystine renal stone formation, Single injections of L-cysteine (0.6-1.5 g/kg) into 4-day-old pups resulted in massive damage to cortical neurons, permanent retinal dystrophy, atrophy of the brain and hyperactivity.

Pathways
Not Available
Pharmacogenomic Effects/ADRs
Not Available

Interactions

Drug Interactions
This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.
Not Available
Food Interactions
Not Available

Products

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Mixture Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing EndRegionImage
Amino-cervCystine (0.354 %) + Inositol (0.83 %) + Sodium propionate (0.50 %) + Racemethionine (0.83 %) + Urea (8.34 %)CreamVaginalMilex, A Coopersurgical Co.1952-12-312007-07-26Canada flag
Bio-Life Pil-Food CapsuleCystine (105 mg) + Biotin (0.2 mg) + Millet seed (20 mg) + Pantothenic acid (25 mg) + Pyridoxine hydrochloride (12.15 mg) + Racemethionine (200 mg) + Riboflavin (1 mg) + Vitamin E (3 IU) + alpha-Lactalbumin (25 mg)CapsuleOralBIO-LIFE MARKETING SDN. BHD.2020-09-08Not applicableMalaysia flag
Distilpure MsmCystine (0.05 g/100g) + Biotin (0.02 g/100g) + Cysteine (0.02 g/100g) + Methionine (0.02 g/100g) + Thiamine chloride (0.02 g/100g)PowderTopicalMbg Inc (Korea Institute of Science Development)2017-09-162018-09-16US flag
Medi Msm (methyl Sulfonyl Methane)Cystine (0.05 g/100g) + Biotin (0.02 g/100g) + Cysteine (0.02 g/100g) + Methionine (0.02 g/100g) + Thiamine chloride (0.02 g/100g)PowderTopicalMbg Inc (Korea Institute of Science Development)2016-09-162016-09-16US flag
Pantogar - KapselnCystine (220 mg) + Calcium pantothenate (60 mg)CapsuleOralMerz Pharmaceuticals1986-10-31Not applicableAustria flag
Unapproved/Other Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing EndRegionImage
Distilpure MsmCystine (0.05 g/100g) + Biotin (0.02 g/100g) + Cysteine (0.02 g/100g) + Methionine (0.02 g/100g) + Thiamine chloride (0.02 g/100g)PowderTopicalMbg Inc (Korea Institute of Science Development)2017-09-162018-09-16US flag
Medi Msm (methyl Sulfonyl Methane)Cystine (0.05 g/100g) + Biotin (0.02 g/100g) + Cysteine (0.02 g/100g) + Methionine (0.02 g/100g) + Thiamine chloride (0.02 g/100g)PowderTopicalMbg Inc (Korea Institute of Science Development)2016-09-162016-09-16US flag

Categories

Drug Categories
Chemical TaxonomyProvided by Classyfire
Description
This compound belongs to the class of organic compounds known as l-cysteine-s-conjugates. These are compounds containing L-cysteine where the thio-group is conjugated.
Kingdom
Organic compounds
Super Class
Organic acids and derivatives
Class
Carboxylic acids and derivatives
Sub Class
Amino acids, peptides, and analogues
Direct Parent
L-cysteine-S-conjugates
Alternative Parents
L-alpha-amino acids / Dicarboxylic acids and derivatives / Dialkyldisulfides / Amino acids / Sulfenyl compounds / Carboxylic acids / Organopnictogen compounds / Organic oxides / Monoalkylamines / Hydrocarbon derivatives
show 1 more
Substituents
Aliphatic acyclic compound / Alpha-amino acid / Amine / Amino acid / Carbonyl group / Carboxylic acid / Dialkyldisulfide / Dicarboxylic acid or derivatives / Hydrocarbon derivative / L-alpha-amino acid
show 12 more
Molecular Framework
Aliphatic acyclic compounds
External Descriptors
non-proteinogenic L-alpha-amino acid, L-cysteine derivative, cystine (CHEBI:16283)
Affected organisms
  • Humans and other mammals

Chemical Identifiers

UNII
48TCX9A1VT
CAS number
56-89-3
InChI Key
LEVWYRKDKASIDU-IMJSIDKUSA-N
InChI
InChI=1S/C6H12N2O4S2/c7-3(5(9)10)1-13-14-2-4(8)6(11)12/h3-4H,1-2,7-8H2,(H,9,10)(H,11,12)/t3-,4-/m0/s1
IUPAC Name
(2R)-2-amino-3-{[(2R)-2-amino-2-carboxyethyl]disulfanyl}propanoic acid
SMILES
N[C@@H](CSSC[C@H](N)C(O)=O)C(O)=O

References

Synthesis Reference

Peter Stehle, Peter Furst, Werner Fekl, "N,N'-bis-L-amino acid-L-cystine-peptide containing amino acid preparations for oral parenteral nutrition." U.S. Patent US4968696, issued February, 1964.

US4968696
General References
Not Available
Human Metabolome Database
HMDB0000192
KEGG Drug
D03636
KEGG Compound
C00491
PubChem Compound
67678
PubChem Substance
46506670
ChemSpider
60997
RxNav
3036
ChEBI
16283
ChEMBL
CHEMBL590540
ZINC
ZINC000001532673
PharmGKB
PA449176
PDBe Ligand
IYY
PDRhealth
PDRhealth Drug Page
Wikipedia
Cystine
PDB Entries
7zkw / 8dkm
MSDS
Download (72 KB)

Clinical Trials

Clinical Trials
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PhaseStatusPurposeConditionsCountStart DateWhy Stopped100+ additional columns
3CompletedTreatmentInfertility1somestatusstop reasonjust information to hide

Pharmacoeconomics

Manufacturers
Not Available
Packagers
  • Mason Distributors
  • Tyson & Associates Inc.
Dosage Forms
FormRouteStrength
CreamVaginal
TabletOral
PowderTopical
CapsuleOral
Prices
Unit descriptionCostUnit
L-alanine 600 mg capsule0.35USD capsule
A-ketoglut acid 500 mg capsule0.31USD capsule
Amino acid capsule0.08USD capsule
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
Patents
Not Available

Properties

State
Solid
Experimental Properties
PropertyValueSource
melting point (°C)260.5 dec °CPhysProp
water solubility190 mg/L (at 20 °C)YALKOWSKY,SH & DANNENFELSER,RM (1992)
logP-5.08CHMELIK,J ET AL. (1991)
pKa1MERCK INDEX (1996)
Predicted Properties
PropertyValueSource
Water Solubility16.8 mg/mLALOGPS
logP-3.2ALOGPS
logP-5.9Chemaxon
logS-1.2ALOGPS
pKa (Strongest Acidic)1.56Chemaxon
pKa (Strongest Basic)9.34Chemaxon
Physiological Charge0Chemaxon
Hydrogen Acceptor Count6Chemaxon
Hydrogen Donor Count4Chemaxon
Polar Surface Area126.64 Å2Chemaxon
Rotatable Bond Count7Chemaxon
Refractivity54.87 m3·mol-1Chemaxon
Polarizability22.77 Å3Chemaxon
Number of Rings0Chemaxon
Bioavailability1Chemaxon
Rule of FiveYesChemaxon
Ghose FilterNoChemaxon
Veber's RuleNoChemaxon
MDDR-like RuleNoChemaxon
Predicted ADMET Features
PropertyValueProbability
Human Intestinal Absorption+0.7309
Blood Brain Barrier-0.5098
Caco-2 permeable-0.7541
P-glycoprotein substrateNon-substrate0.606
P-glycoprotein inhibitor INon-inhibitor0.9749
P-glycoprotein inhibitor IINon-inhibitor1.0
Renal organic cation transporterNon-inhibitor0.9521
CYP450 2C9 substrateNon-substrate0.9012
CYP450 2D6 substrateNon-substrate0.8371
CYP450 3A4 substrateNon-substrate0.8232
CYP450 1A2 substrateNon-inhibitor0.8924
CYP450 2C9 inhibitorNon-inhibitor0.8968
CYP450 2D6 inhibitorNon-inhibitor0.9149
CYP450 2C19 inhibitorNon-inhibitor0.9101
CYP450 3A4 inhibitorNon-inhibitor0.6103
CYP450 inhibitory promiscuityLow CYP Inhibitory Promiscuity0.995
Ames testNon AMES toxic0.5429
CarcinogenicityNon-carcinogens0.781
BiodegradationReady biodegradable0.628
Rat acute toxicity2.0474 LD50, mol/kg Not applicable
hERG inhibition (predictor I)Weak inhibitor0.9839
hERG inhibition (predictor II)Non-inhibitor0.9707
ADMET data is predicted using admetSAR, a free tool for evaluating chemical ADMET properties. (23092397)

Spectra

Mass Spec (NIST)
Download (2.96 KB)
Spectra
SpectrumSpectrum TypeSplash Key
GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)GC-MSsplash10-00kb-0950000000-a803bc05843192dd737d
GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)GC-MSsplash10-00kb-0940000000-f82b905ef9e7c9e47552
GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)GC-MSsplash10-00di-9850000000-49b9a52a8387d6d6f272
GC-MS Spectrum - GC-MS (4 TMS)GC-MSsplash10-014j-1970000000-9576699202733d4fd7ed
Predicted GC-MS Spectrum - GC-MSPredicted GC-MSsplash10-0076-9400000000-211575093fd775db5d54
GC-MS Spectrum - GC-EI-TOFGC-MSsplash10-00kb-0950000000-a803bc05843192dd737d
GC-MS Spectrum - GC-EI-TOFGC-MSsplash10-00di-9850000000-49b9a52a8387d6d6f272
GC-MS Spectrum - GC-MSGC-MSsplash10-014j-1970000000-9576699202733d4fd7ed
GC-MS Spectrum - GC-EI-TOFGC-MSsplash10-00kb-0930000000-7cb336c3f272fd0fcd6b
GC-MS Spectrum - GC-EI-TOFGC-MSsplash10-00kb-0920000000-1c414573da4bbe4a66dd
Mass Spectrum (Electron Ionization)MSsplash10-000x-9000000000-ae0f516d71e6a49fe52f
MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)LC-MS/MSsplash10-0006-0890000000-51a32ee40240e45646bc
MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)LC-MS/MSsplash10-00di-9700000000-4054924ddfb90f120e67
MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)LC-MS/MSsplash10-00di-9100000000-085d45702e61de0f8e4d
Predicted MS/MS Spectrum - 10V, Positive (Annotated)Predicted LC-MS/MSsplash10-0006-0690000000-c9ed2825bb2ff21cc983
Predicted MS/MS Spectrum - 10V, Negative (Annotated)Predicted LC-MS/MSsplash10-0jb9-4910000000-82233412f15aeaea9583
Predicted MS/MS Spectrum - 20V, Positive (Annotated)Predicted LC-MS/MSsplash10-0adi-4900000000-678dec1ee523397c5b6b
Predicted MS/MS Spectrum - 20V, Negative (Annotated)Predicted LC-MS/MSsplash10-03di-9200000000-99006742305a7fcc28a9
Predicted MS/MS Spectrum - 40V, Negative (Annotated)Predicted LC-MS/MSsplash10-03fr-9000000000-7ac9eceb20672a9428cb
Predicted MS/MS Spectrum - 40V, Positive (Annotated)Predicted LC-MS/MSsplash10-0a4i-3900000000-1948235e4c1d1e5185fc
1H NMR Spectrum1D NMRNot Applicable
13C NMR Spectrum1D NMRNot Applicable
1H NMR Spectrum1D NMRNot Applicable
1H NMR Spectrum1D NMRNot Applicable
1H NMR Spectrum1D NMRNot Applicable
13C NMR Spectrum1D NMRNot Applicable
Predicted 1H NMR Spectrum1D NMRNot Applicable
Predicted 13C NMR Spectrum1D NMRNot Applicable
[1H,1H] 2D NMR Spectrum2D NMRNot Applicable
[1H,13C] 2D NMR Spectrum2D NMRNot Applicable
Chromatographic Properties
Collision Cross Sections (CCS)
AdductCCS Value (Å2)Source typeSource
[M-H]-156.5186047
predicted
DarkChem Lite v0.1.0
[M-H]-156.5333047
predicted
DarkChem Lite v0.1.0
[M-H]-156.6452047
predicted
DarkChem Lite v0.1.0
[M-H]-156.6999047
predicted
DarkChem Lite v0.1.0
[M-H]-140.53249
predicted
DeepCCS 1.0 (2019)
[M+H]+156.6924047
predicted
DarkChem Lite v0.1.0
[M+H]+148.1189348
predicted
DarkChem Standard v0.1.0
[M+H]+156.6622047
predicted
DarkChem Lite v0.1.0
[M+H]+156.4245047
predicted
DarkChem Lite v0.1.0
[M+H]+142.92804
predicted
DeepCCS 1.0 (2019)
[M+Na]+155.7846047
predicted
DarkChem Lite v0.1.0
[M+Na]+155.6069047
predicted
DarkChem Lite v0.1.0
[M+Na]+155.7902047
predicted
DarkChem Lite v0.1.0
[M+Na]+155.8434047
predicted
DarkChem Lite v0.1.0
[M+Na]+149.29123
predicted
DeepCCS 1.0 (2019)

Targets

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insights and accelerate drug research.
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Kind
Protein
Organism
Humans
Pharmacological action
Unknown
General Function
Heterodimer with SLC3A2, that functions as an antiporter by mediating the exchange of extracellular anionic L-cystine and intracellular L-glutamate across the cellular plasma membrane (PubMed:11133847, PubMed:11417227, PubMed:14722095, PubMed:15151999, PubMed:34880232, PubMed:35245456, PubMed:35352032). Provides L-cystine for the maintenance of the redox balance between extracellular L-cystine and L-cysteine and for the maintenance of the intracellular levels of glutathione that is essential for cells protection from oxidative stress (By similarity). The transport is sodium-independent, electroneutral with a stoichiometry of 1:1, and is drove by the high intracellular concentration of L-glutamate and the intracellular reduction of L-cystine (PubMed:11133847, PubMed:11417227). In addition, mediates the import of L-kynurenine leading to anti-ferroptotic signaling propagation required to maintain L-cystine and glutathione homeostasis (PubMed:35245456). Moreover, mediates N-acetyl-L-cysteine uptake into the placenta leading to subsequently down-regulation of pathways associated with oxidative stress, inflammation and apoptosis (PubMed:34120018). In vitro can also transport L-aspartate (PubMed:11417227). May participate in astrocyte and meningeal cell proliferation during development and can provide neuroprotection by promoting glutathione synthesis and delivery from non-neuronal cells such as astrocytes and meningeal cells to immature neurons (By similarity). Controls the production of pheomelanin pigment directly (By similarity)
Specific Function
cystine
Gene Name
SLC7A11
Uniprot ID
Q9UPY5
Uniprot Name
Cystine/glutamate transporter
Molecular Weight
55422.44 Da
References
  1. Tomi M, Hosoya K, Takanaga H, Ohtsuki S, Terasaki T: Induction of xCT gene expression and L-cystine transport activity by diethyl maleate at the inner blood-retinal barrier. Invest Ophthalmol Vis Sci. 2002 Mar;43(3):774-9. [Article]
  2. Hosoya K, Tomi M, Ohtsuki S, Takanaga H, Saeki S, Kanai Y, Endou H, Naito M, Tsuruo T, Terasaki T: Enhancement of L-cystine transport activity and its relation to xCT gene induction at the blood-brain barrier by diethyl maleate treatment. J Pharmacol Exp Ther. 2002 Jul;302(1):225-31. [Article]
  3. Ruiz E, Siow RC, Bartlett SR, Jenner AM, Sato H, Bannai S, Mann GE: Vitamin C inhibits diethylmaleate-induced L-cystine transport in human vascular smooth muscle cells. Free Radic Biol Med. 2003 Jan 1;34(1):103-10. [Article]
  4. Bridges CC, Zalups RK: Cystine and glutamate transport in renal epithelial cells transfected with human system x(-) (c). Kidney Int. 2005 Aug;68(2):653-64. [Article]
  5. Tomi M, Funaki T, Abukawa H, Katayama K, Kondo T, Ohtsuki S, Ueda M, Obinata M, Terasaki T, Hosoya K: Expression and regulation of L-cystine transporter, system xc-, in the newly developed rat retinal Muller cell line (TR-MUL). Glia. 2003 Sep;43(3):208-17. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
General Function
Cystine/H(+) symporter that mediates export of cystine, the oxidized dimer of cysteine, from lysosomes (PubMed:11689434, PubMed:15128704, PubMed:18337546, PubMed:22232659, PubMed:29467429, PubMed:33208952, PubMed:36113465). Plays an important role in melanin synthesis by catalyzing cystine export from melanosomes, possibly by inhibiting pheomelanin synthesis (PubMed:22649030). In addition to cystine export, also acts as a positive regulator of mTORC1 signaling in kidney proximal tubular cells, via interactions with components of the v-ATPase and Ragulator complexes (PubMed:36113465). Also involved in small GTPase-regulated vesicle trafficking and lysosomal localization of LAMP2A, independently of cystine transporter activity (By similarity)
Specific Function
L-cystine transmembrane transporter activity
Gene Name
CTNS
Uniprot ID
O60931
Uniprot Name
Cystinosin
Molecular Weight
41737.6 Da
References
  1. Kalatzis V, Cherqui S, Antignac C, Gasnier B: Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter. EMBO J. 2001 Nov 1;20(21):5940-9. [Article]

Transporters

Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Inhibitor
General Function
Sodium- and proton-independent thyroid hormones and aromatic acids transporter (PubMed:11827462, PubMed:18337592, PubMed:28754537). Mediates both uptake and efflux of 3,5,3'-triiodothyronine (T3) and 3,5,3',5'-tetraiodothyronine (T4) with high affinity, suggesting a role in the homeostasis of thyroid hormone levels (PubMed:18337592). Responsible for low affinity bidirectional transport of the aromatic amino acids, such as phenylalanine, tyrosine, tryptophan and L-3,4-dihydroxyphenylalanine (L-dopa) (PubMed:11827462, PubMed:28754537). Plays an important role in homeostasis of aromatic amino acids (By similarity)
Specific Function
amino acid transmembrane transporter activity
Gene Name
SLC16A10
Uniprot ID
Q8TF71
Uniprot Name
Monocarboxylate transporter 10
Molecular Weight
55492.07 Da
References
  1. Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
General Function
Associates with SLC3A1 to form a functional transporter complex that mediates the electrogenic exchange between cationic amino acids and neutral amino acids, with a stoichiometry of 1:1 (PubMed:16825196, PubMed:32494597, PubMed:32817565, PubMed:8663357). Has system b(0,+)-like activity with high affinity for extracellular cationic amino acids and L-cystine and lower affinity for intracellular neutral amino acids (PubMed:16825196, PubMed:32494597, PubMed:8663357). Substrate exchange is driven by high concentration of intracellular neutral amino acids and the intracellular reduction of L-cystine to L-cysteine (PubMed:8663357). Required for reabsorption of L-cystine and dibasic amino acids across the brush border membrane in renal proximal tubules
Specific Function
antiporter activity
Gene Name
SLC7A9
Uniprot ID
P82251
Uniprot Name
b(0,+)-type amino acid transporter 1
Molecular Weight
53480.81 Da
References
  1. Rius M, Sala L, Chillaron J: The role of N-glycans and the C-terminal loop of the subunit rBAT in the biogenesis of the cystinuria-associated transporter. Biochem J. 2016 Feb 1;473(3):233-44. doi: 10.1042/BJ20150846. Epub 2015 Nov 4. [Article]
  2. Chillaron J, Font-Llitjos M, Fort J, Zorzano A, Goldfarb DS, Nunes V, Palacin M: Pathophysiology and treatment of cystinuria. Nat Rev Nephrol. 2010 Jul;6(7):424-34. doi: 10.1038/nrneph.2010.69. Epub 2010 Jun 1. [Article]
  3. Nagamori S, Wiriyasermkul P, Guarch ME, Okuyama H, Nakagomi S, Tadagaki K, Nishinaka Y, Bodoy S, Takafuji K, Okuda S, Kurokawa J, Ohgaki R, Nunes V, Palacin M, Kanai Y: Novel cystine transporter in renal proximal tubule identified as a missing partner of cystinuria-related plasma membrane protein rBAT/SLC3A1. Proc Natl Acad Sci U S A. 2016 Jan 19;113(3):775-80. doi: 10.1073/pnas.1519959113. Epub 2016 Jan 6. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
General Function
Acts as a chaperone that facilitates biogenesis and trafficking of functional transporter heteromers to the plasma membrane (By similarity) (PubMed:10588648, PubMed:11318953, PubMed:16609684, PubMed:16825196, PubMed:32494597, PubMed:32817565, PubMed:7686906, PubMed:8486766, PubMed:8663184, PubMed:8663357). Associates with SLC7A9 to form a functional transporter complex that mediates the electrogenic exchange between cationic amino acids and neutral amino acids, with a stoichiometry of 1:1. SLC7A9-SLC3A1 transporter has system b(0,+)-like activity with high affinity for extracellular cationic amino acids and L-cystine and lower affinity for intracellular neutral amino acids. Substrate exchange is driven by high concentration of intracellular neutral amino acids and the intracellular reduction of L-cystine to L-cysteine. SLC7A9-SLC3A1 acts as a major transporter for reabsorption of L-cystine and dibasic amino acids across the brush border membrane in early proximal tubules (PubMed:10588648, PubMed:11318953, PubMed:16609684, PubMed:16825196, PubMed:32494597, PubMed:32817565, PubMed:7686906, PubMed:8486766, PubMed:8663184, PubMed:8663357). Associates with SLC7A13 to form a functional complex that transports anionic and neutral amino acids via exchange or facilitated diffusion. SLC7A13-SLC3A1 may act as a major transporter for L-cystine in late proximal tubules, ensuring its reabsorption from the luminal fluid in exchange for cytosolic L-glutamate or L-aspartate (By similarity)
Specific Function
amino acid transmembrane transporter activity
Gene Name
SLC3A1
Uniprot ID
Q07837
Uniprot Name
Amino acid transporter heavy chain SLC3A1
Molecular Weight
78851.4 Da
References
  1. Nagamori S, Wiriyasermkul P, Guarch ME, Okuyama H, Nakagomi S, Tadagaki K, Nishinaka Y, Bodoy S, Takafuji K, Okuda S, Kurokawa J, Ohgaki R, Nunes V, Palacin M, Kanai Y: Novel cystine transporter in renal proximal tubule identified as a missing partner of cystinuria-related plasma membrane protein rBAT/SLC3A1. Proc Natl Acad Sci U S A. 2016 Jan 19;113(3):775-80. doi: 10.1073/pnas.1519959113. Epub 2016 Jan 6. [Article]
  2. Chillaron J, Font-Llitjos M, Fort J, Zorzano A, Goldfarb DS, Nunes V, Palacin M: Pathophysiology and treatment of cystinuria. Nat Rev Nephrol. 2010 Jul;6(7):424-34. doi: 10.1038/nrneph.2010.69. Epub 2010 Jun 1. [Article]

Drug created at June 13, 2005 13:24 / Updated at August 02, 2024 07:22