ACADSB Antibody

Code CSB-PA001128GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
ACADSB
Alternative Names
2 MEBCAD antibody; 2 methyl branched chain acyl CoA dehydrogenase antibody; 2 methylbutyryl CoA dehydrogenase antibody; 2 methylbutyryl coenzyme A dehydrogenase antibody; 2-MEBCAD antibody; 2-methyl branched chain acyl-CoA dehydrogenase antibody; 2-methylbutyryl-CoA dehydrogenase antibody; 2-methylbutyryl-coenzyme A dehydrogenase antibody; ACAD7 antibody; ACADSB antibody; ACDSB_HUMAN antibody; acyl CoA dehydrogenase; short/branched chain antibody; acyl Coenzyme A dehydrogenase short branched chain antibody; mitochondrial antibody; OTTHUMP00000020685 antibody; OTTHUMP00000046795 antibody; SBCAD antibody; Short/branched chain specific acyl-CoA dehydrogenase antibody; short/branched chain specific acyl-CoA dehydrogenase; mitochondrial antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human ACADSB
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,IHC
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
Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA. Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway. Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug.
Gene References into Functions
  1. the c.1165 A>G mutation before knowing whether the optimal screening cut-off would minimize true positives or false negatives for SBCADD associated with this mutation. PMID: 23712021
  2. Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
  3. These findings confirm that SBCAD deficiency can be identified through newborn screening by acylcarnitine analysis. PMID: 20547083
  4. Differences between the rat and human enzyme at positions 383, 222, and 220 alter substrate specificity without affecting substrate binding. PMID: 12855692
  5. Identification and characterization of an IVS3+3A>G mutation (c.303+3A>G) in the SBCAD gene, and provide evidence that this mutation is disease-causing. PMID: 16317551
  6. Results examine the mechanistic basis for dysfunction of the common variant short-chain acyl-CoA dehydrogenases and demonstrate that mutations can have a large impact on the redox properties of the enzyme. PMID: 16331963
  7. Results indicate that substrate redox activation occurs in short-chain acyl-CoA dehydrogenase leading to a large enzyme midpoint potential shift. PMID: 16331964

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Involvement in disease
Short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD)
Subcellular Location
Mitochondrion matrix.
Protein Families
Acyl-CoA dehydrogenase family
Tissue Specificity
Ubiquitously expressed.
Database Links

HGNC: 91

OMIM: 600301

KEGG: hsa:36

STRING: 9606.ENSP00000357873

UniGene: Hs.81934

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