SAN TA C RUZ BI OTEC HNOL OG Y, INC . PDPr (S-17): sc-248229

BACKGROUND PRODUCT PDPr (pyruvate dehydrogenase phosphatase regulatory subunit) is an 879 Each vial contains 200 µg IgG in 1.0 ml of PBS with < 0.1% sodium azide amino acid mitochondrial matrix that belongs to the GcvT family. and 0.1% gelatin. While it forms a heterodimer with PDPc, PDPr decreases the sensitivity of Blocking peptide available for competition studies, sc-248229 P, (100 µg PDPc to magnesium ions, which can be reversed by the polyamine spermine. pep tide in 0.5 ml PBS containing < 0.1% sodium azide and 0.2% BSA). The gene that encodes PDPr consists of more than 47,000 bases and maps to human 16q22.1. Encoding over 900 and consisting of APPLICATIONS approximately 90 million base pairs, makes up nearly 3% of the and is associated with a variety of genetic disorders, PDPr (S-17) is recommended for detection of PDPr of human origin, such as giant axonal neuropathy, Rubinstein-Taybi syndrome and Crohn’s dis - 4930402E16Rik of mouse origin and the corresponding rat homolog by ease. An association with systemic lupus erythematosis and a number of Western Blotting (starting dilution 1:200, dilution range 1:100-1:1000), other autoimmune disorders within the pericentromeric region of chromo - immunoprecipitation [1-2 µg per 100-500 µg of total protein (1 ml of cell some 16 has led to the identification of SLC5A11 as a potential autoimmune lysate)], immunofluorescence (starting dilution 1:50, dilution range 1:50- modifier. 1:500) and solid phase ELISA (starting dilution 1:30, dilution range 1:30- 1:3000). REFERENCES PDPr (S-17) is also recommended for detection of PDPr in additional species, 1. Mentzer, W.C., et al. 1977. An unusual form of chronic neutropenia in a including equine, canine, bovine and avian. father and daughter with hypogammaglobulinaemia. Br. J. Haematol. 36: Suitable for use as control antibody for PDPr siRNA (h): sc-93518, PDPr 313-322. siRNA (m): sc-140021, PDPr shRNA Plasmid (h): sc-93518-SH, PDPr shRNA 2. Baraitser, M. and Preece, M.A. 1983. The Rubinstein-Taybi syndrome: Plasmid (m): sc-140021-SH, PDPr shRNA (h) Lentiviral Particles: sc-93518-V occurrence in two sets of identical twins. Clin. Genet. 23: 318-320. and PDPr shRNA (m) Lentiviral Particles: sc-140021-V. 3. Breuning, M.H., et al. 1993. Rubinstein-Taybi syndrome caused by submi - Molecular Weight of PDPr: 99 kDa. croscopic deletions within 16p13.3. Am. J. Hum. Genet. 52: 249-254. Positive Controls: RAW 264.7 whole cell lysate: sc-2211. 4. Lawson, J.E., et al. 1997. Cloning, expression, and properties of the regu - latory subunit of bovine pyruvate dehydrogenase phosphatase. J. Biol. RECOMMENDED SECONDARY REAGENTS Chem. 272: 31625-31629. To ensure optimal results, the following support (secondary) reagents are 5. Bomont, P., et al. 2000. The encoding gigaxonin, a new member of recommended: 1) Western Blotting: use donkey anti-goat IgG-HRP: sc-2020 the cytoskeletal BTB/kelch repeat family, is mutated in giant axonal neu - (dilution range: 1:2000-1:100,000) or Cruz Marker™ compatible donkey ropathy. Nat. Genet. 26: 370-374. anti- goat IgG-HRP: sc-2033 (dilution range: 1:2000-1:5000), Cruz Marker™ Molecular Weight Standards: sc-2035, TBS Blotto A Blocking Reagent: 6. Kuhlenbäumer, G., et al. 2002. Giant axonal neuropathy (GAN): case report sc-2333 and Western Blotting Luminol Reagent: sc-2048. 2) Immunoprecip- and two novel mutations in the gigaxonin gene. Neurology 58: 1273-1276. itation: use Protein A/G PLUS-Agarose: sc-2003 (0.5 ml agarose/2.0 ml). 7. Sugden, M.C. and Holness, M.J. 2003. Recent advances in mechanisms 3) Immunofluorescence: use donkey anti-goat IgG-FITC: sc-2024 (dilution regulating oxidation at the level of the pyruvate dehydrogenase range: 1:100-1:400) or donkey anti-goat IgG-TR: sc-2783 (dilution range: complex by PDKs. Am. J. Physiol. Endocrinol. Metab. 284: E855-E862. 1:100-1:400) with UltraCruz™ Mounting Medium: sc-24941. 8. Cho, J.H. 2004. Advances in the genetics of inflammatory bowel disease. DATA Curr. Gastroenterol. Rep. 6: 467-473.

CHROMOSOMAL LOCATION 132 K – Genetic locus: PDPR (human) mapping to 16q22.1; Pdpr (mouse) mapping to < PDPr 8 E1. 90K–

SOURCE 55K– PDPr (S-17) is an affinity purified goat polyclonal antibody raised against a peptide mapping within an internal region of PDPr of human origin. PDPr (S-17): sc-248229. Westernblot analysisof PDPr expressioninRAW 264.7 whole cell lysate. STORAGE Store at 4° C, **DO NOT FREEZE**. Stable for one year from the date of RESEARCH USE shipment. Non-hazardous. No MSDS required. For research use only, not for use in diagnostic procedures.

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