ACAD8 Antibody

Code CSB-PA001123GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
ACAD8
Alternative Names
ACAD 8 antibody; ACAD-8 antibody; ACAD8 antibody; ACAD8_HUMAN antibody; Activator-recruited cofactor 42 kDa component antibody; Acyl CoA dehydrogenase family member 8 antibody; Acyl Coenzyme A dehydrogenase family member 8 antibody; Acyl-CoA dehydrogenase family member 8 antibody; ARC42 antibody; FLJ22590 antibody; IBD antibody; Isobutyryl-CoA dehydrogenase antibody; Isobutyryl-CoA dehydrogenase; mitochondrial antibody; mitochondrial antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human ACAD8
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Isobutyryl-CoA dehydrogenase which catalyzes one of the steps of the valine catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA.
Gene References into Functions
  1. we discovered a novel c.1156_1158delCAG mutation in ACAD8 in patients with isobutyryl-CoA dehydrogenase deficiency , and investigated the mutation spectrum of ACAD8. PMID: 24635911
  2. first enzymatic and molecular confirmation of a deficiency of this enzyme in a patient PMID: 12359132
Involvement in disease
Isobutyryl-CoA dehydrogenase deficiency (IBDD)
Subcellular Location
Mitochondrion.
Protein Families
Acyl-CoA dehydrogenase family
Tissue Specificity
Detected at comparable levels in all tissues examined (heart, lung, brain, skeletal muscle, pancreas and placenta). Weakly expressed in liver and kidney.
Database Links

HGNC: 87

OMIM: 604773

KEGG: hsa:27034

STRING: 9606.ENSP00000281182

UniGene: Hs.14791

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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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