Isovaleryl Coenzyme A Dehydrogenase Recombinant Protein | IVD recombinant protein

Recombinant Isovaleryl Coenzyme A Dehydrogenase (IVD)

Cat. Num. AAA2034371

• Gene Names: IVD; ACAD2
• Reactivity: Mouse
• Applications: SDS-Page, Western Blot, ELISA, Immunoprecipitation
• Purity: >95%
• Synonyms: Isovaleryl Coenzyme A Dehydrogenase; Recombinant Isovaleryl Coenzyme A Dehydrogenase (IVD); ACAD2; IVD recombinant protein

• Host: E Coli
• Reactivity: Mouse
• Purity/Purification: >95%
• Sequence Length: 423

• Applicable Applications for IVD recombinant protein: SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Fragment: Met1~Phe235
• Tag: Prokaryotic expression
• Preparation and Storage: Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.; Stability Test: The thermal stability is described by the loss rate of the target protein. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss of this protein is less than 5% within the expiration date under appropriate storage condition.

SDS-Page

NCBI and Uniprot Product Information

• NCBI GI #: 16877964
• NCBI GeneID: 3712
• Molecular Weight: 29.4kDa
• NCBI Official Full Name: Isovaleryl Coenzyme A dehydrogenase
• NCBI Official Synonym Full Names: isovaleryl-CoA dehydrogenase
• NCBI Official Symbol: IVD
• NCBI Official Synonym Symbols: ACAD2
• NCBI Protein Information: isovaleryl-CoA dehydrogenase, mitochondrial
• UniProt Protein Name: Isovaleryl-CoA dehydrogenase, mitochondrial
• Protein Family: Isovaleryl-CoA dehydrogenase
• UniProt Gene Name: IVD
• UniProt Synonym Gene Names: IVD
• UniProt Entry Name: IVD_HUMAN

NCBI Description

Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009]

Uniprot Description

IVD: Defects in IVD are the cause of isovaleric acidemia (IVA). IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. Belongs to the acyl-CoA dehydrogenase family.

Protein type: Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; EC 1.3.8.4

Chromosomal Location of Human Ortholog: 15q14-q15

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: acyl-CoA binding; electron carrier activity; FAD binding; isovaleryl-CoA dehydrogenase activity

Biological Process: branched chain family amino acid catabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; leucine catabolic process; lipid homeostasis

Disease: Isovaleric Acidemia