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Human recombinant Methylmalonyl-CoA epimerase (from cells)

Artikel-Nr: (PRSI92-387)

Lieferant: ProSci Inc.

Beschreibung: Methylmalonyl-CoA epimerase, mitochondrial（MCEE）is an enzyme which belongs to the glyoxalase I family. It converts (S)-methylmalonyl-CoA to the (R) form, catalyses the following chemical reaction: (R)-methylmalonyl-CoA (S)-methylmalonyl-CoA. It plays an important role in the catabolism of fatty acids with odd-length carbon chains. This protein deficiency is an autosomal recessive inborn error of AA metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria can appear in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma.

UOM: 1 * 50 µG

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N/A Stück

Anti-CTTNBL1 Rabbit Polyclonal Antibody

Artikel-Nr: (PRSI6215)

Lieferant: ProSci Inc.

Beschreibung: CTTNBL1 Antibody: The Beta-catenin-like protein 1 (CTNNBL1) contains an acidic domain, a putative bipartite nuclear localization signal, a nuclear export signal, a leucine-isoleucine zipper, and phosphorylation motifs, as well as Armadillo/beta-catenin-like repeats. Transient expression of CTNNBL1 resulted in translocation to the nucleus and apoptosis, suggesting it may be involved in the apoptotic pathway. CTNNBL1 interacts with the Prp19 complex of the spliceosome and the Ig class switching enzyme activation-induced deaminase (AID) and had been suggested to play a role in antibody-diversification and class switching, but recent studies have shown CTNNBL1 to be dispensable for Ig class switch recombination. Other studies have identified CTTNBL1 as a novel gene for obesity.

UOM: 1 * 100 µG

Aktion

N/A Stück

Anti-BCKDHA Rabbit Polyclonal Antibody

Artikel-Nr: (PRSI31-325)

Lieferant: ProSci Inc.

Beschreibung: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998 [PubMed 9582350]). The BCKD complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331). E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene (MIM 248611) encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.

UOM: 1 * 50 µG

Aktion