Anti-PNP Antibody Picoband® (monoclonal, 2H10) Fluoro647 Conjugated

The PNP gene encodes purine nucleoside phosphorylase, an enzyme that catalyzes the reversible phosphorolysis of the purine nucleosides and deoxynucleosides inosine, guanosine, deoxyinosine, and deoxyguanosine. It is presented results from gene dosage studies consistent with assignment of the PNP locus to band 14q13. PNP is expressed in most tissues, with markedly greater expression in lymphoid tissues. Genetic deficiencies of PNP result in severely compromised Tlymphocyte function and neurologic dysfunction.

A synthetic peptide corresponding to a sequence in the middle region of human PNP, different from the related mouse sequence by six amino acids, and from the related rat sequence by five amino acids.

ATP-dependent specificity component of the Clp protease complex. Hydrolyzes ATP (PubMed:28874591) . Targets specific substrates for degradation by the Clp complex (PubMed:11923310, PubMed:22710082) . Can perform chaperone functions in the absence of CLPP. Enhances the DNA-binding activity of TFAM and is required for maintaining a normal mitochondrial nucleoid structure (PubMed:22841477) . ATP-dependent unfoldase that stimulates the incorporation of the pyridoxal phosphate cofactor into 5-aminolevulinate synthase, thereby activating 5-aminolevulinate (ALA) synthesis, the first step in heme biosynthesis (PubMed:28874591) . Important for efficient erythropoiesis through upregulation of heme biosynthesis (PubMed:25957689, PubMed:28874591) .

1. Williams, S. R., Goddard, J. M., Martin, D. W., Jr. Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization. Nucleic Acids Res. 12: 5779-5787, 1984. 2. Frecker, M., Dallaire, L., Young, S. R., Chen, G. C. C., Simpson, N. E. Confirmation of regional assignment of nucleoside phosphorylase (NP) on chromosome 14 by gene dosage studies. Hum. Genet. 45: 167-173, 1978. 3. Markert, M. L., Finkel, B. D., McLaughlin, T. M., Watson, T. J., Collard, H. R., McMahon, C. P., Andrews, L. G., Barrett, M. J., Ward, F. E. Mutations in purine nucleoside phosphorylase deficiency. Hum. Mutat. 9: 118-121, 1997.