Anti-Slc25a1 Picoband® Antibody Fluoro488 Conjugated

Tricarboxylate transport protein, mitochondrial, also known as tricarboxylate carrier protein and citrate transport protein (CTP), is a protein that in humans is encoded by the SLC25A1 gene. It is mapped to 22q11.21. This gene encodes a member of the mitochondrial carrier subfamily of solute carrier proteins. Members of this family include nuclear-encoded transporters that translocate small metabolites across the mitochondrial membrane. This protein regulates the movement of citrate across the inner membranes of the mitochondria. Mutations in this gene have been associated with combined D-2- and L-2-hydroxyglutaric aciduria. Pseudogenes of this gene have been identified on chromosomes 7, 11, 16, and 19. 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. Chaouch, A., Porcelli, V., Cox, D., Edvardson, S., Scarcia, P., De Grassi, A., Pierri, C. L., Cossins, J., Laval, S. H., Griffin, H., Muller, J. S., Evangelista, T., and 13 others. Mutations in the mitochondrial citrate carrier SLC25A1 are associated with impaired neuromuscular transmission. J. Neuromusc. Dis. 1: 75-90, 2014. 2. Edvardson, S., Porcelli, V., Jalas, C., Sioferman, D., Kelner, Y., Shaaq, A., Korman, S. H., Pierri, C. L., Scarcia, P., Fraenkel, N. D., Segel, R., Schechter, A., Frumkin, A., Pines, O., Saada, A., Palmieri, ., Elpeleg, O. Agenesis of corpus callosum and optic nerve hypoplasia due to mutations in SLC25A1 encoding the mitochondrial citrate transporter. J. Med. Genet. 50: 240-245, 2013. 3. Goldmuntz, E., Wang, Z., Roe, B. A., Budarf, M. L. Cloning, genomic organization, and chromosomal localization of human citrate transport protein to the DiGeorge/velocardiofacial syndrome minimal critical region. Genomics 33: 271-276, 1996.