Anti-PDIA6 Antibody Picoband® Fluoro647 Conjugated

This gene encodes a member of the disulfide isomerase (PDI) family of endoplasmic reticulum (ER) proteins that catalyze protein folding and thiol-disulfide interchange reactions. The encoded protein has an N-terminal ER-signal sequence, two catalytically active thioredoxin (TRX) domains, a TRX-like domain, and a C-terminal ER-retention sequence. This protein inhibits the aggregation of misfolded proteins and exhibits both isomerase and chaperone activity. Alternative splicing results in multiple transcript variants encoding different isoforms.

Lon protease homolog, mitochondrial; LONHs; Lon protease-like protein; LONP; Mitochondrial ATP-dependent protease Lon; Serine protease 15; LONP1; PRSS15

ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, helicase Twinkle (TWNK) and the large ribosomal subunit protein bL32m. bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not.

1. Chaudhuri, M. M., Tonin, P. N., Lewis, W. H., Srinivasan, P. R. The gene for a novel protein, a member of the protein disulphide isomerase/Form I phosphoinositide-specific phospholipase C family, is amplified in hydroxyurea-resistant cells. Biochem. J. 281: 645-650, 1992. 2. Hayano, T., Kikuchi, M. Cloning and sequencing of the cDNA encoding human P5. Gene 164: 377-378, 1995. 3. Hoshijima, K., Metherall, J. E., Grunwald, D. J. A protein disulfide isomerase expressed in the embryonic midline is required for left/right asymmetries. Genes Dev. 16: 2518-2529, 2002.