Acyl-Coenzyme A Dehydrogenase 8 Recombinant Protein | ACAD8 recombinant protein

Recombinant Human Acyl-Coenzyme A Dehydrogenase 8

Cat. Num. AAA142435

• Gene Names: ACAD8; ARC42; ACAD-8
• Purity: Greater than 95% as determined by SDS-PAGE.
• Synonyms: Acyl-Coenzyme A Dehydrogenase 8; Recombinant Human Acyl-Coenzyme A Dehydrogenase 8; ACAD8 Human; Acyl-Coenzyme A Dehydrogenase 8 Human Recombinant; Acyl-CoA dehydrogenase family member 8 mitochondrial; ACAD-8; Isobutyryl-CoA dehydrogenase; Activator-recruited cofactor 42 kDa component; ARC42; FLJ22590; ACAD8 recombinant protein

• Host: E Coli
• Purity/Purification: Greater than 95% as determined by SDS-PAGE.
• Form/Format: The ACAD8 solution contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 1mM DTT and 30% glycerol.; Sterile Filtered clear solution.
• Sequence: MGSSHHHHHH SSGLVPRGSH MGSLVQTGHR SLTSCIDPSM GLNEEQKEFQ KVAFDFAARE MAPNMAEWDQ KELFPVDVMR KAAQLGFGGV YIQTDVGGSG LSRLDTSVIF EALATGCTST TAYISIHNMC AWMIDSFGNE EQRHKFCPPL CTMEKFASYC LTEPGSGSDA ASLLTSAKKQ GDHYILNGSK AFISGAGESD IYVVMCRTGG PGPKGISCIV VEKGTPGLSF GKKEKKVGWN SQPTRAVIFE DCAVPVANRI GSEGQGFLIA VRGLNGGRIN IASCSLGAAH ASVILTRDHL NVRKQFGEPL ASNQYLQFTL ADMATRLVAA RLMVRNAAVA LQEERKDAVA LCSMAKLFAT DECFAICNQA LQMHGGYGYL KDYAVQQYVR DSRVHQILEG SNEVMRILIS RSLLQE
• Sequence Length: 415

• Preparation and Storage: Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.

Related Product Information for ACAD8 recombinant protein

Description: ACAD8 Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 416 amino acids (23-415) and having a molecular mass of 45.1kDa.ACAD8 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Categories/Family for ACAD8 recombinant protein

Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 7656849
• NCBI GeneID: 27034
• NCBI Accession #: NP_055199.1
• NCBI GenBank Nucleotide #: NM_014384.2
• UniProt Accession #: Q9UKU7; Q6ZWP6; Q9BUS8; B7Z5W4
• Molecular Weight: 38,359 Da
• NCBI Official Full Name: isobutyryl-CoA dehydrogenase, mitochondrial
• NCBI Official Synonym Full Names: acyl-CoA dehydrogenase family, member 8
• NCBI Official Symbol: ACAD8
• NCBI Official Synonym Symbols: ARC42; ACAD-8
• NCBI Protein Information: isobutyryl-CoA dehydrogenase, mitochondrial; activator-recruited cofactor 42 kDa component; acyl-Coenzyme A dehydrogenase family, member 8
• UniProt Protein Name: Isobutyryl-CoA dehydrogenase, mitochondrial
• Protein Family: Isobutyryl-CoA dehydrogenase
• UniProt Gene Name: ACAD8
• UniProt Synonym Gene Names: ARC42; IBD; ARC42; ACAD-8
• UniProt Entry Name: ACAD8_HUMAN

NCBI Description

This gene encodes a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. The encoded protein is a mitochondrial enzyme that functions in catabolism of the branched-chain amino acid valine. Defects in this gene are the cause of isobutyryl-CoA dehydrogenase deficiency.[provided by RefSeq, Nov 2009]

Uniprot Description

ACAD8: Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex. Defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency (IBDD). The symptoms of IBDD generally appear until late in infancy or in childhood and can include poor feeding and growth (failure to thrive), a weakened and enlarged heart (dilated cardiomyopathy), seizures, and low numbers of red blood cells (anemia). Belongs to the acyl-CoA dehydrogenase family.

Protein type: Oxidoreductase; EC 1.3.99.-; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 11q25

Cellular Component: mitochondrial matrix

Molecular Function: acyl-CoA dehydrogenase activity; FAD binding

Biological Process: valine catabolic process; transcription, DNA-dependent; regulation of transcription, DNA-dependent; lipid metabolic process; branched chain family amino acid catabolic process

Disease: Isobutyryl-coa Dehydrogenase Deficiency