Anti-HADHA Antibody Picoband®

SKU: A03666-2

Size: 100 μg

Storage: 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.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

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

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

Gene Name: HADHA

Specificity:

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.

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

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

Purification: Immunogen affinity purified.

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

Reference: 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.

Uniprot ID: P40939

Host: Rabbit

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

Conjugate:

Cross Reactivity:

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 79 kDa

Calculated Molecular Weight: 83 kDa

Gene ID: 3030

Protein Name: Trifunctional enzyme subunit alpha, mitochondrial

Gene Full Name: hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha

Synonyms: 78 kDa gastrin binding protein; ECHA; HADH; HADHA; LCHAD; MTPA; TP ALPHA