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Mouse Anti-IVD Recombinant Antibody (1F1) (CBMAB-I0116-YY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
1F1
Antibody Isotype
IgG2b
Application
FC, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2b
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
1% BSA, pH 7.3, 50% glycerol
Preservative
0.02% sodium azide
Concentration
0.94 mg/ml
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Isovaleryl-CoA Dehydrogenase
Introduction
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants have been found for this gene.
Entrez Gene ID
3712
UniProt ID
P26440
Alternative Names
Isovaleryl-CoA Dehydrogenase
Function
Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268).
To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA (PubMed:7640268).
Biological Process
Branched-chain amino acid catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Fatty acid beta-oxidation using acyl-CoA dehydrogenaseManual Assertion Based On ExperimentIDA:BHF-UCL
Leucine catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
Isovaleric acidemia (IVA):
A metabolic disorder characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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