PPCS Antibody

Phosphopantothenoylcysteine Synthetase (PPCS) is an enzyme that plays a crucial role in the biosynthesis of coenzyme A (CoA) from pantothenic acid (vitamin B5). This enzyme is essential in both prokaryotic and eukaryotic organisms, highlighting its universal importance in cellular metabolism .

PPCS catalyzes the second step in the CoA biosynthesis pathway. Specifically, it converts 4’-phosphopantothenate to 4’-phosphopantothenoylcysteine by conjugating cysteine to 4’-phosphopantothenate . This reaction is crucial for the subsequent steps that ultimately lead to the formation of CoA, a vital cofactor involved in various biochemical processes, including the synthesis and oxidation of fatty acids and the metabolism of carbohydrates and amino acids .

PPCS belongs to the family of ligases, specifically those forming carbon-nitrogen bonds as acid-D-amino-acid ligases (peptide synthases). The enzyme’s systematic name is ®-4’-phosphopantothenate:L-cysteine ligase (EC 6.3.2.5) .

The gene encoding PPCS in humans is located on chromosome 1 (1p34.2), while in mice, it is located on chromosome 4 (4 D2.1) . The human PPCS gene is identified by several aliases, including CMD2C and COAB .

Mouse anti-human PPCS antibodies are used in various research applications to study the expression and function of PPCS in human cells. These antibodies are typically generated by immunizing mice with human PPCS protein, leading to the production of specific antibodies that can be harvested and purified for experimental use.

Mutations or dysregulation of the PPCS gene can lead to metabolic disorders. For instance, PPCS has been associated with conditions such as dilated cardiomyopathy, a disease characterized by the enlargement and weakening of the heart muscle . Understanding the role of PPCS in these conditions can provide insights into potential therapeutic targets.