catalog number :
MBS203892
products type :
Recombinant Protein
products full name :
GSS, 1-474aa, Human, His tag, E Coli
products short name :
GSS
products name syn :
Glutathione synthetase; GSHS; GSH synthetase
other names :
glutathione synthetase; Glutathione synthetase; glutathione synthetase; GSH synthetase; GSH-S; epididymis secretory sperm binding protein Li 64p; epididymis secretory sperm binding protein Li 88n; glutathione synthase; glutathione synthetase; Glutathione synthase
other gene names :
GSS; GSS; GSHS; HEL-S-64p; HEL-S-88n; GSH synthetase; GSH-S
uniprot entry name :
GSHB_HUMAN
sequence :
MGSSHHHHQH SSGLVPRGSH MATNWGSLLQ DKQQLEELAR QAVDRALAEG VLLRTSQEPT SSEVVSYAPF TLFPSLVPSA LLEQAYAVQM DFNLLVDAVS QNAAFLEQTL SSTIKQDDFT ARLFDIHKQV LKEGIAQTVF LGLNRSDYMF QRSADGSPAL KQIEINTISA SFGGLASRTP AVHRHVLSVL SKTKEAGKIL SNNPSKGLAL GIAKAWELYG SPNALVLLIA QEKERNIFDQRAIENELLAR NIHVIRRTFE DISEKGSLDQ DRRLFVDGQE IAVVYFRDGY MPRQYSLQNW EARLLLERSH AAKCPDIATQ LAGTKKVQQE LSRPGMLEML LPGQPEAVAR LRATFAGLYS LDVGEEGDQA IAEALAAPSR FVLKPQREGG GNNLYGEEMV QALKQLKDSE ERASYILMEK IEPEPFENCL LRPGSPARVV QCISELGIFG VYVRQEKTLV MNKHVGHLLR TKAIEHADGG VAAGVAVLDN PYPV
purity :
> 95% by SDS - PAGE
form :
Liquid. In 20mM Tris-HCl buffer (pH 8.0) containing 1mM DTT, 10% glycerol
concentration :
1 mg/ml (determined by Bradford assay)
storage stability :
Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.
tested application :
SDS-PAGE
other info1 :
Antigen Species: Human. Tag: His-tag
other info2 :
Expression System: E Coli
products categories :
Neuroscience
products description :
Glutathione synthetase, also known GSS, is the second enzyme in the glutathione biosynthesis pathway. It catalyses the condensation of gamma-glutamylcysteine and glycine, to form glutathione. Defects in GSS are the cause of glutathione synthetase deficiency (GSS deficiency); also known as 5-oxoprolinuria or pyroglutamic aciduria. It is a severe form characterized by an increased rate of hemolysis and defective function of the central nervous system. Recombinant human GSS protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.
products references :
Polekhina G., et al. (1999) EMBO J. 18:3204-3213. Huanq Z A., et al. (2000) Biochim Biophys Acta. 1493:48-55.
ncbi gb acc num :
NM_000178.2
ncbi mol weight :
54.5 kDa (494aa) confirmed by MALDI-TOF
ncbi pathways :
Biological Oxidations Pathway (105698); Defective AHCY Causes Hypermethioninemia With S-adenosylhomocysteine Hydrolase Deficiency (HMAHCHD) Pathway (1127639); Defective GCLC Causes Hemolytic Anemia Due To Gamma-glutamylcysteine Synthetase Deficiency (HAGGSD) Pathway (1127658); Defective GGT1 Causes Glutathionuria (GLUTH) Pathway (1127659); Defective GSS Causes Glutathione Synthetase Deficiency (GSS Deficiency) Pathway (1127660); Defective MAT1A Causes Methionine Adenosyltransferase Deficiency (MATD) Pathway (1127663); Defective OPLAH Causes 5-oxoprolinase Deficiency (OPLAHD) Pathway (1127661); Defective SLC35D1 Causes Schneckenbecken Dysplasia (SCHBCKD) Pathway (1127665); Defective TPMT Causes Thiopurine S-methyltransferase Deficiency (TPMT Deficiency) Pathway (1127666); Defective UGT1A1 Causes Hyperbilirubinemia Pathway (1127667)
ncbi summary :
Glutathione is important for a variety of biological functions, including protection of cells from oxidative damage by free radicals, detoxification of xenobiotics, and membrane transport. The protein encoded by this gene functions as a homodimer to catalyze the second step of glutathione biosynthesis, which is the ATP-dependent conversion of gamma-L-glutamyl-L-cysteine to glutathione. Defects in this gene are a cause of glutathione synthetase deficiency. [provided by RefSeq, Jul 2008]
uniprot summary :
GSS: Defects in GSS are the cause of glutathione synthetase deficiency (GSS deficiency); also known as 5- oxoprolinuria or pyroglutamic aciduria. It is a severe form characterized by an increased rate of hemolysis and defective function of the central nervous system. Defects in GSS are the cause of glutathione synthetase deficiency of erythrocytes (GLUSYNDE)[MIM:231900]. Glutathione synthetase deficiency of erythrocytes is a mild form causing hemolytic anemia. Belongs to the eukaryotic GSH synthase family. Protein type: Ligase; EC 6.3.2.3; Other Amino Acids Metabolism - glutathione. Chromosomal Location of Human Ortholog: 20q11.2. Cellular Component: cytosol. Molecular Function: protein homodimerization activity; glutathione binding; glycine binding; magnesium ion binding; glutathione synthase activity; ATP binding. Biological Process: response to nutrient levels; amino acid metabolic process; nervous system development; response to cadmium ion; xenobiotic metabolic process; glutathione biosynthetic process; response to oxidative stress; response to amino acid stimulus; aging. Disease: Glutathione Synthetase Deficiency; Glutathione Synthetase Deficiency Of Erythrocytes, Hemolytic Anemia Due To