| ELK.No | ES13199 |
| Product name | SARDH rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB;ELISA;IHC |
| Other name | |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 1757 |
| Human Swiss-Prot | Q9UL12 |
| Source | Rabbit |
| Isotype | IgG |
| Target | SARDH |
| Fields | >>Glycine, serine and threonine metabolism;>>Metabolic pathways |
| Gene name | SARDH DMGDHL1 |
| Protein name | SARDH |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 192166 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q99LB7 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 114123 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q64380 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human SARDH AA range: 279-329 |
| Specificity | This antibody detects endogenous levels of SARDH at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000;IHC-p 1:50-300; ELISA 2000-20000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 101kD |
| Observed band (KD) | |
| Background | This gene encodes an enzyme localized to the mitochondrial matrix which catalyzes the oxidative demethylation of sarcosine. This enzyme is distinct from another mitochondrial matrix enzyme, dimethylglycine dehydrogenase, which catalyzes a reaction resulting in the formation of sarcosine. Mutations in this gene are associated with sarcosinemia. Alternatively spliced transcript variants have been described. [provided by RefSeq, Oct 2008], |
| Function | catalytic activity:Sarcosine + acceptor + H(2)O = glycine + formaldehyde + reduced acceptor.,cofactor:Binds 1 FAD covalently per monomer.,disease:Defects in SARDH are a ause of sarcosinemia [MIM:268900]. Sarcosinemia is a metabolic disorder characterized by an increased concentration of sarcosine in plasma and urine due to sarcosine dehydrogenase deficiency. Prevalence has been estimated at 1:28'000 to 1:350'000 in newborn screening programs. Sarcosinemia is most probably a benign condition without significant clinical problems. It is transmitted in an autosomal recessive manner.,pathway:Amine and polyamine degradation; sarcosine degradation; formaldehyde and glycine from sarcosine: step 1/1.,similarity:Belongs to the gcvT family., |
| Subcellular location | Mitochondrion matrix . |
| Expression | Expressed in pancreas, liver and kidney. |


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