product summary
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company name :
MyBioSource
product type :
protein
product name :
GPD1L, 1-351aa, Human, His tag, E Coli
catalog :
MBS204445
quantity :
0.1 mg
price :
310 USD
more info or order :
product information
catalog number :
MBS204445
products type :
Recombinant Protein
products full name :
GPD1L, 1-351aa, Human, His tag, E Coli
products short name :
GPD1L
products name syn :
Glycerol-3-phosphate dehydrogenase 1-like protein; GPD1-L
other names :
glycerol-3-phosphate dehydrogenase 1-like protein; Glycerol-3-phosphate dehydrogenase 1-like protein; glycerol-3-phosphate dehydrogenase 1-like protein; glycerol-3-phosphate dehydrogenase 1-like
products gene name :
GPD1L
other gene names :
GPD1L; GPD1L; GPD1-L; KIAA0089; GPD1-L
uniprot entry name :
GPD1L_HUMAN
sequence length :
351
sequence :
MGSSHHHHHH SSGLVPRGSH MAAAPLKVCI VGSGNWGSAV AKIIGNNVKK LQKFASTVKM WVFEETVNGR KLTDIINNDH ENVKYLPGHK LPENVVAMSN LSEAVQDADL LVFVIPHQFI HRICDEITGR VPKKALGITL IKGIDEGPEG LKLISDIIRE KMGIDISVLM GANIANEVAA EKFCETTIGS KVMENGLLFK ELLQTPNFRI TVVDDADTVE LCGALKNIVA VGAGFCDGLR CGDNTKAAVI RLGLMEMIAF ARIFCKGQVS TATFLESCGV ADLITTCYGG RNRRVAEAFA RTGKTIEELE KEMLNGQKLQ GPQTSAEVYR ILKQKGLLDK FPLFTAVYQI CYESRPVQEM LSCLQSHPEH T
purity :
> 95% by SDS - PAGE
form :
Liquid. 20mM Tris-HCl buffer (pH8.0) containing 20% glycerol, 1mM DTT
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 :
Metabolism
products description :
GPD1L, also known as glycerol-3-phosphate dehydrogenase 1-like protein, converts sn-glycerol 3-phosphate to glycerone phosphate. This protein is found in the cytoplasm, associated with the plasma membrane, where it binds the sodium channel, voltage-gated, type V, alpha subunit (SCN5A). Mutations in GPD1L are the cause of sudden infant death syndrome (SIDS) and Brugada syndrome type 2, an autosomal dominant tachyarrhythmia. Recombinant human GPD1L protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography.
products references :
Valdivia C.R., et al. (2009) Am. J. Physiol. 297:H1446-H1452. Van Norstrand D.W., et al. (2007) Circulation. 116:2253-2259
ncbi gi num :
24307999
ncbi acc num :
NP_055956
ncbi gb acc num :
NM_015141.3
ncbi mol weight :
40.6 kDa (371aa), confirmed by MALDI-TOF
ncbi pathways :
Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (160977); Glycerophospholipid Biosynthesis Pathway (645313); Glycerophospholipid Metabolism Pathway (82989); Glycerophospholipid Metabolism Pathway (364); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Phospholipid Metabolism Pathway (645312); SIDS Susceptibility Pathways (198901); Synthesis Of PA Pathway (645314); Triglyceride Biosynthesis Pathway (106114)
ncbi summary :
The protein encoded by this gene catalyzes the conversion of sn-glycerol 3-phosphate to glycerone phosphate. The encoded protein is found in the cytoplasm, associated with the plasma membrane, where it binds the sodium channel, voltage-gated, type V, alpha subunit (SCN5A). Defects in this gene are a cause of Brugada syndrome type 2 (BRS2) as well as sudden infant death syndrome (SIDS). [provided by RefSeq, Jul 2010]
uniprot summary :
GPD1L: Plays a role in regulating cardiac sodium current; decreased enzymatic activity with resulting increased levels of glycerol 3-phosphate activating the DPD1L-dependent SCN5A phosphorylation pathway, may ultimately lead to decreased sodium current; cardiac sodium current may also be reduced due to alterations of NAD(H) balance induced by DPD1L. Defects in GPD1L are the cause of Brugada syndrome type 2 (BRGDA2). An autosomal dominant tachyarrhythmia characterized by right bundle branch block and ST segment elevation on an electrocardiogram (ECG). It can cause the ventricles to beat so fast that the blood is prevented from circulating efficiently in the body. When this situation occurs (called ventricular fibrillation), the individual will faint and may die in a few minutes if the heart is not reset. Defects in GPD1L are a cause of sudden infant death syndrome (SIDS). SIDS is the sudden death of an infant younger than 1 year that remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene, and review of clinical history. Pathophysiologic mechanisms for SIDS may include respiratory dysfunction, cardiac dysrhythmias, cardiorespiratory instability, and inborn errors of metabolism, but definitive pathogenic mechanisms precipitating an infant sudden death remain elusive. Belongs to the NAD-dependent glycerol-3-phosphate dehydrogenase family. Protein type: Oxidoreductase; Lipid Metabolism - glycerophospholipid; EC 1.1.1.8. Chromosomal Location of Human Ortholog: 3p22.3. Cellular Component: glycerol-3-phosphate dehydrogenase complex; plasma membrane; cytosol. Molecular Function: glycerol-3-phosphate dehydrogenase (NAD+) activity; protein homodimerization activity; sodium channel regulator activity; NAD binding. Biological Process: NADH metabolic process; regulation of heart rate; NAD metabolic process; phospholipid metabolic process; glycerophospholipid biosynthetic process; carbohydrate metabolic process; negative regulation of peptidyl-serine phosphorylation; phosphatidic acid biosynthetic process; triacylglycerol biosynthetic process; cellular lipid metabolic process; glycerol-3-phosphate catabolic process. Disease: Brugada Syndrome 2
size1 :
0.1 mg
price1 :
310 USD
size2 :
0.5 mg
price2 :
720
more info or order :
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com
1-888-627-0165
headquarters: USA
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