Anti-PYCR1 Antibody Picoband® Fluoro488 Conjugated

Pyrroline-5-carboxylate reductase 1, mitochondrial is an enzyme that in humans is encoded by the PYCR1 gene. This gene encodes an enzyme that catalyzes the NAD (P) H-dependent conversion of pyrroline-5-carboxylate to proline. This enzyme may also play a physiologic role in the generation of NADP (+) in some cell types. The protein forms a homopolymer and localizes to the mitochondrion. Alternative splicing results in multiple transcript variants.

Tricarboxylate transport protein, mitochondrial; Citrate transport protein; CTP; Solute carrier family 25 member 1; Tricarboxylate carrier protein; SLC25A1; SLC20A3

Detected in brain. Detected at very much lower levels in heart, lung, placenta and skeletal muscle. Highly expressed in cerebellum, but also found in frontal cortex, hippocampus and basal ganglia.

Citrate transporter that mediates the exchange of mitochondrial citrate for cytosolic malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis- but not trans-aconitate and to a lesser extend maleate and succinate. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD+ for the glycolytic pathway. Required for proper neuromuscular junction formation (Probable) .

1. Al-Gazali, L. I., Sztriha, L., Skaff, F., Haas, D. Gerodermia osteodysplastica and wrinkly skin syndrome: are they the same? Am. J. Med. Genet. 101: 213-220, 2001. 2. De Ingeniis, J., Ratnikov, B., Richardson, A. D., Scott, D. A., Aza-Blanc, P., De, S. K., Kazanov, M., Pellecchia, M., Ronai, Z., Osterman, A. L., Smith, J. W. Functional specialization in proline biosynthesis of melanoma. PLoS One 7: e45190, 2012. Note: Electronic Article. 3. Dougherty, K. M., Brandriss, M. C., Valle, D. Cloning human pyrroline-5-carboxylate reductase cDNA by complementation in Saccharomyces cerevisiae. J. Biol. Chem. 267: 871-875, 1992.