Abcam, ab156763, Anti-H-protein antibody [OTI3G1]

Size: 100µL
Mouse Monoclonal H-protein antibody. Suitable for WB, IHC-P, ICC/IF and reacts with Human, African green monkey samples. Cited in 1 publication. Immunogen corresponding to Recombinant Full Length Protein corresponding to Human MYBPH.
Key facts
Host species:Mouse,
Clonality:Monoclonal,
Clone number:OTI3G1,
Isotype:IgG2a,
Carrier free:No,
Reacts with:Human, African green monkey,
Applications:ICC/IF, WB, IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Full Length Protein corresponding to Human MYBPH.Q13203

Product details:
The clone number has been updated from 3G1 to OTI3G1, both clone numbers name the same clone.

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.3Preservative: 0.02% Sodium azideConstituents: PBS, 50% Glycerol (glycerin, glycerine), 1% BSA, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
The H-protein also known as GCSh (Glycine Cleavage System H) protein plays an important role in the glycine cleavage system. It has a molecular mass of approximately 14 kDa. This protein is expressed in various tissues with significant expression in the liver and kidneys. Mechanically H-protein functions as a carrier protein facilitating the transfer of a methylamine group during the glycine decarboxylation process. This action is important in the conversion of glycine a non-essential amino acid to carbon dioxide ammonia NADH and methylene tetrahydrofolate.
Biological function summary
The function of H-protein is essential for cellular energy production and provision of one-carbon units for metabolism. H-protein is part of the glycine cleavage complex which includes P-protein T-protein and L-protein. This complex collectively breaks down glycine an important step for energy generation and maintaining low levels of toxic glycine in mitochondria. This process also contributes to the pool of one-carbon units necessary for various biosynthetic pathways impacting nucleotide synthesis and methylation reactions.
Pathways
The glycine cleavage system which includes H-protein is integrated into two key biological pathways: amino acid metabolism and mitochondrial energy production. In amino acid metabolism it collaborates with other proteins like P-protein to facilitate the breakdown of glycine thereby regulating levels of this amino acid. In mitochondrial energy production the activity of H-protein ensures the removal of excess glycine which can affect mitochondrial function and energy balance if accumulated. This dynamic further connects it to wider metabolic cycles impacting overall cellular health and efficiency.
Disruptions in H-protein function are linked to non-ketotic hyperglycinemia and possibly a few cases of metabolic disorders involving glycine. Non-ketotic hyperglycinemia results from mutations affecting the glycine cleavage system including defective activities in GCS-related proteins like P-protein which leads to elevated glycine levels and neurological issues. Studies also suggest that altered H-protein activity may contribute to other mitochondrial disorders as its efficient function is important for maintaining mitochondrial health thereby indirectly connecting with proteins involved in mitochondrial dynamics and metabolism.