Anti-HADHA Antibody Picoband®, Cy3 Conjugate

Boster Bio Anti-HADHA Antibody Picoband® catalog # A03666-2. Tested in WB, IHC, ICC/IF, Flow Cytometry, ELISA applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Product Specifications

Background

Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit is a protein that in humans is encoded by the HADHA gene. This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.

Gene Name

HADHA

Gene ID

3030

UniProt

P40939

Host

Rabbit

Reactivity

Human,Mouse,Rat

Immunogen

E.coli-derived human HADHA recombinant protein (Position: R20-N758). Human HADHA shares 86.6% and 95.2% amino acid (aa) sequence identity with mouse and rat HADHA, respectively.

Clonality

Polyclonal

Applications

ELISA,Flow Cytometry,IF,IHC,ICC,WB

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Components

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

References & Citations

1. Brackett, J. C., Sims, H. F., Rinaldo, P., Shapiro, S., Powell, C. K., Bennett, M. J., Strauss, A. W. Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency. J. Clin. Invest. 95: 2076-2082, 1995. 2. Craig, I., Tolley, E., Bobrow, M. A preliminary analysis of the segregation of human hydroxyacyl coenzyme A dehydrogenase in human-mouse somatic cell hybrids. Birth Defects Orig. Art. Ser. XII(7): 114-117, 1976. 3. Dionisi Vici, C., Burlina, A. B., Bertini, E., Bachmann, C., Mazziotta, M. R. M., Zacchello, F., Sabetta, G., Hale, D. E. Progressive neuropathy and recurrent myoglobinuria in a child with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. J. Pediat. 118: 744-746, 1991.

Storage Conditions

At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Calculated Molecular Weight

83 kDa

Observed Molecular Weight

79 kDa

Applications Notes

Western blot, 0.1-0.25 μg/ml, Human, Mouse, Rat Immunohistochemistry, 2-5 μg/ml, Human Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human ELISA, 0.1-0.5 μg/ml, -