protein

Recombinant Human Acyl-Coenzyme A Dehydrogenase 8

MBS142435

0.005 mg

140 USD

Recombinant Protein

Recombinant Human Acyl-Coenzyme A Dehydrogenase 8

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

isobutyryl-CoA dehydrogenase, mitochondrial; Isobutyryl-CoA dehydrogenase, mitochondrial; isobutyryl-CoA dehydrogenase, mitochondrial; activator-recruited cofactor 42 kDa component; acyl-Coenzyme A dehydrogenase family, member 8; acyl-CoA dehydrogenase family, member 8; Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8

ACAD8

ACAD8; ACAD8; ARC42; ACAD-8; ARC42; IBD; ARC42; ACAD-8

ACAD8_HUMAN

E Coli

415

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

Greater than 95% as determined by SDS-PAGE.

The ACAD8 solution contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 1mM DTT and 30% glycerol. Sterile Filtered clear solution.

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.

ENZYMES; Enzymes; Dehydrogenase

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.

7656849

NP_055199.1

NM_014384.2

Q9UKU7

38,359 Da

Branched-chain Amino Acid Catabolism Pathway (106179); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Valine, Leucine And Isoleucine Degradation Pathway (82952); Valine, Leucine And Isoleucine Degradation Pathway (316); Valine Degradation Pathway (142387)

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]

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

0.005 mg

140 USD

0.02 mg

205

1 mg

2665