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ACAD8 Antibody, FITC conjugated

Datasheet
Code CSB-PA892457LC01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) ACAD8 Polyclonal antibody
Uniprot No.
Q9UKU7
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
Immunogen
Recombinant Human Isobutyryl-CoA dehydrogenase, mitochondrial protein (201-415AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
FITC
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Troubleshooting and FAQs
Antibody 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.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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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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