Anti-GCSH Polyclonal Antibody for WB, IF/ICC, ELISA - P23434

Core Product Specifications and Parameters

Biological Background: GCSH Function and Localization

• GCSH encodes the mitochondrial glycine cleavage system H protein, a lipoic acid-containing component essential for glycine degradation.
• As part of the glycine cleavage system, GCSH shuttles the methylamine group from glycine decarboxylase (P protein) to aminomethyltransferase (T protein), facilitating one-carbon metabolism.
• GCSH plays a pivotal role in protein lipoylation, donating lipoyl groups to key metabolic enzymes such as the E2 components of pyruvate dehydrogenase (DLAT) and 2-oxoglutarate dehydrogenase (DLST) complexes (PubMed:36190515). Related references: PMID:36190515
• The protein localizes to the mitochondrial matrix, consistent with its function in mitochondrial energy metabolism.
• GCSH undergoes lipoylation, a post-translational modification critical for its cofactor function, and contains a transit peptide for mitochondrial import.
• Mutations in GCSH are associated with nonketotic hyperglycinemia (NKH) and neurodegenerative disorders, underscoring its clinical importance.
• It is also known as lipoic acid-containing protein, reflecting its covalently bound lipoate moiety.

Experimental Guidance and Technical Tips

• The antibody is raised against full-length human GCSH recombinant protein; its polyclonal nature may recognize multiple epitopes but also potential non-specific bands.
• For WB, the predicted molecular weight is 19 kDa; a band near this size suggests specific detection. Use appropriate loading controls.
• For IF/ICC, fixation and permeabilization conditions may need optimization to detect mitochondrial GCSH; co-staining with mitochondrial markers is recommended.
• Cross-reactivity with mouse and rat is reported; validate in the relevant species and tissue type.
• ELISA applications may include direct or sandwich formats; titration and pairing with detection antibodies are advised.
• Given the protein's role in glycine metabolism, verify expression in tissues like liver or brain where glycine cleavage is active.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio specializes in providing high-quality IVD raw materials, including validated antibody pairs and bulk ancillary reagents, to support diagnostic development. For glycine metabolism research and related disorders, we offer a comprehensive range of recombinant proteins, antibodies, and technical consultation. Inquire about custom sourcing for rare targets and bulk orders to accelerate your assay development.