Anti-GMPPB monoclonal antibody (DMABT-H13850)

Mouse anti-Human GMPPB monoclonal antibody for WB, IHC, sELISA, ELISA Datasheet

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Specifications


Host Species
Mouse
Antibody Isotype
IgG2b
Clone
3C6
Species Reactivity
Human
Immunogen
GMPPB (NP_037466,2a.a. ~ 110 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Conjugate
Unconjugated

Target


Alternative Names
GMPPB; GDP-mannose pyrophosphorylase B; mannose-1-phosphate guanyltransferase beta; KIAA1851; mannose-1-phosphate guanylyltransferase; GTP-mannose-1-phosphate guanylyltransferase beta
Entrez Gene ID
UniProt ID

Product Background


Gene summary
GMPPB (GDP-Mannose Pyrophosphorylase B) is a Protein Coding gene. Diseases associated with GMPPB include muscular dystrophy-dystroglycanopathy, type c, 14 and muscular dystrophy-dystroglycanopathy, type a, 14. Among its related pathways are Metabolism and Transport to the Golgi and subsequent modification. GO annotations related to this gene include GTP binding and mannose-1-phosphate guanylyltransferase activity. An important paralog of this gene is GMPPA. This gene is thought to encode a GDP-mannose pyrophosphorylase. The encoded protein catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose, a reaction involved in the production of N-linked oligosaccharides. Alternatively spliced transcript variants encoding distinct isoforms have been described.
Antigen Description
Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A14 (MDDGA14) [MIM:615350]: An autosomal recessive disorder characterized by congenital muscular dystrophy associated with brain anomalies, eye malformations, and profound mental retardation. The disorder includes a severe form designated as Walker-Warburg syndrome and a less severe phenotype known as muscle-eye-brain disease. MDDGA14 features include increased muscle tone, microcephaly, cleft palate, feeding difficulties, severe muscle weakness, sensorineural hearing loss, cerebellar hypoplasia, ataxia, and retinal dysfunction. Note=The disease is caused by mutations affecting the gene represented in this entry. Muscular dystrophy-dystroglycanopathy congenital with mental retardation B14 (MDDGB14) [MIM:615351]: A congenital muscular dystrophy characterized by severe muscle weakness apparent in infancy and mental retardation. Some patients may have additional features, such as microcephaly, cardiac dysfunction, seizures, or cerebellar hypoplasia. Muscular dystrophy-dystroglycanopathy limb-girdle C14 (MDDGC14) [MIM:615352]: An autosomal recessive form of muscular dystrophy characterized by mild proximal muscle weakness with onset in early childhood. Some patients may have additional features, such as mild intellectual disability or seizures. Mannose-1-phosphate guanyltransferase beta is an enzyme that in humans is encoded by the GMPPB gene. 0The function about GMPPB antigen include GTP binding; mannose-1-phosphate guanylyltransferase activity; nucleotide binding; nucleotidyltransferase activity; transferase activity.
Pathway
Amino sugar and nucleotide sugar metabolism, organism-specific biosystem; Amino sugar and nucleotide sugar metabolism, conserved biosystem; Asparagine N-linked glycosylation, organism-specific biosystem; Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein, organism-specific biosystem; Fructose and mannose metabolism, organism-specific biosystem; Fructose and mannose metabolism, conserved biosystem; Metabolic pathways, organism-spe.

Citations


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References


STROEHER, UH; KARAGEORGOS, LE; et al. A PUTATIVE PATHWAY FOR PEROSAMINE BIOSYNTHESIS IS THE FIRST FUNCTION ENCODED WITHIN THE RFB REGION OF VIBRIO-CHOLERAE O1. GENE 166:33-42(1995).
Weuster-Botz, D; Altenbach-Rehm, J; et al. Parallel substrate feeding and pH-control in shaking-flasks. BIOCHEMICAL ENGINEERING JOURNAL 7:163-170(2001).

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