Anti-PMPCA/INPP5 Antibody Picoband®

SKU: A09623-1

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

Gene Name: PMPCA

Specificity:

Background: Mitochondrial-processing peptidase subunit alpha is an enzyme that in humans is encoded by the PMPCA gene. The protein encoded by this gene is found in the mitochondrion, where it represents the alpha subunit of a proteolytic heterodimer. This heterodimer is responsible for cleaving the transit peptide from nuclear-encoded mitochondrial proteins. Defects in this gene are a cause of spinocerebellar ataxia, autosomal recessive 2.

Immunogen: E.coli-derived human PMPCA/INPP5 recombinant protein (Position: R99-R525).

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. Choquet, K., Zurita-Rendon, O., La Piana, R., Yang, S., Dicaire, M.-J., Care4Rare Consortium, Boycott, K. M., Majewski, J., Shoubridge, E. A., Brais, B., Tetreault, M. Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA. (Letter) Brain 139: e19, 2016. Note: Electronic Article. 2. Hartz, P. A. Personal Communication. Baltimore, Md. 9/28/2009. 3. Jobling, R. K., Assoum, M., Gakh, O., Blaser, S., Raiman, J. A., Mignot, C., Roze, E., Durr, A., Brice, A., Levy, N., Prasad, C., Paton, T., and 11 others. PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia. Brain 138: 1505-1517, 2015.

Uniprot ID: Q10713

Host: Rabbit

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

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 50-58 kDa

Calculated Molecular Weight:

Gene ID: 23203

Protein Name: Mitochondrial-processing peptidase subunit alpha

Gene Full Name: peptidase, mitochondrial processing subunit alpha

Synonyms: Alpha MPP; INPP5E; KIAA0123; MPPA; P 55; PMPCA