Anti-PNPT1 Antibody Picoband®

SKU: A05460-1

Size: 100 μg

Storage: Store 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 freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

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

Application Details: Western blot, 0.1-0.25μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human, Rat
Immunocytochemistry/Immunofluorescence, 4μg/ml, Human
Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human
ELISA, 0.1-0.5μg/ml, -

Gene Name: PNPT1

Specificity:

Background: The protein encoded by this gene belongs to the evolutionary conserved polynucleotide phosphorylase family comprised of phosphate dependent 3'-to-5' exoribonucleases implicated in RNA processing and degradation. This enzyme is predominantly localized in the mitochondrial intermembrane space and is involved in import of RNA to mitochondria. Mutations in this gene have been associated with combined oxidative phosphorylation deficiency-13 and autosomal recessive nonsyndromic deafness-70. Related pseudogenes are found on chromosomes 3 and 7.

Immunogen: E.coli-derived human PNPT1 recombinant protein (Position: M1-Q257).

Clonality: Polyclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Liu, X., Fu, R., Pan, Y., Meza-Sosa, K. F., Zhang, Z., Lieberman, J. PNPT1 release from mitochondria during apoptosis triggers decay of poly(A) RNAs. Cell 174: 187-201, 2018. 2. Vedrenne, V., Gowher, A., De Lonlay, P., Nitschke, P., Serre, V., Boddaert, N., Altuzarra, C., Mager-Heckel, A.-M., Chretien, F., Entelis, N., Munnich, A., Tarassov, I., Rotig, A. Mutation in PNPT1, which encodes a polyribonucleotide nucleotidyltransferase, impairs RNA import into mitochondria and causes respiratory-chain deficiency. Am. J. Hum. Genet. 91: 912-918, 2012. 3. von Ameln, S., Wang, G., Boulouiz, R., Rutherford, M. A., Smith, G. M., Li, Y., Pogoda, H.-M., Nurnberg, G., Stiller, B., Volk, A. E., Borck, G., Hong, J. S., and 12 others. A mutation in PNPT1, encoding mitochondrial-RNA-import protein PNPase, causes hereditary hearing loss. Am. J. Hum. Genet. 91: 919-927, 2012.

Uniprot ID: Q8TCS8

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: 86 kDa

Calculated Molecular Weight: 39411 MW

Gene ID: 87178

Protein Name: Polyribonucleotide nucleotidyltransferase 1, mitochondrial

Gene Full Name: polyribonucleotide nucleotidyltransferase 1

Synonyms: 3 5 RNA exonuclease OLD35; old 35; OLD35; PNPASE; PNPase 1; PNPase old 35; PNPT1; Polynucleotide phosphorylase 1