anti-GDP-Mannose Pyrophosphorylase B (GMPPB) Antikörper

GMPPB is thought to encode a GDP-mannose pyrophosphorylase. Zusätzlich bieten wir Ihnen GDP-Mannose Pyrophosphorylase B Proteine (9) und und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
GMPPB 29925 Q9Y5P6
GMPPB 331026 Q8BTZ7
GMPPB 363145  
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Top anti-GDP-Mannose Pyrophosphorylase B Antikörper auf antikoerper-online.de

Showing 10 out of 46 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Lieferzeit Preis Details
Rind (Kuh) Kaninchen Unkonjugiert WB WB Suggested Anti-Gmppb Antibody   Titration: 1.0 ug/ml   Positive Control: Mouse Pancreas 100 μL 2 bis 3 Tage
$319.00
Details
Rind (Kuh) Kaninchen Unkonjugiert IHC, WB Human kidney 100 μL 2 bis 3 Tage
$229.00
Details
Rind (Kuh) Kaninchen Unkonjugiert IHC, IHC (p), WB 100 μL 11 bis 14 Tage
$493.17
Details
Fruchtfliege Kaninchen Unkonjugiert WB 100 μL 11 bis 14 Tage
$581.17
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Human Maus Unkonjugiert ELISA, IHC, IHC (p), WB 100 μg 11 bis 14 Tage
$551.83
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Human Maus Unkonjugiert IHC (p), ELISA, WB Immunoperoxidase of monoclonal antibody to GMPPB on formalin-fixed paraffin-embedded human adrenal gland. [antibody concentration 3 ug/ml] GMPPB monoclonal antibody (M07), clone 2B5 Western Blot analysis of GMPPB expression in HepG2 . 100 μg 11 bis 12 Tage
$440.00
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Human Kaninchen Unkonjugiert WB Western Blot at 1:2000 dilution Lane 1: human pancreas lysate Lane 2: mouse pancreas lysate Lysates/proteins at 20 ug per lane. 200 μL 2 bis 3 Tage
$515.63
Details
Maus Kaninchen Unkonjugiert WB Western Blot at 1:4000 dilution + mouse pancreas lysate Lysates/proteins at 20 ug per lane. 200 μL 2 bis 3 Tage
$515.63
Details
Human Kaninchen Unkonjugiert IHC, WB GMPPB antibody was used for immunohistochemistry at a concentration of 4-8 ug/ml to stain Epithelial cells of renal tubule (arrows) in Human Kidney. Magnification is at 400X GMPPB antibody was used for immunohistochemistry at a concentration of 4-8 ug/ml. Magnification is at 400X 100 μg 9 bis 11 Tage
$369.29
Details
Human Kaninchen Unkonjugiert IHC, ELISA, WB 100 μL 2 bis 3 Tage
$446.88
Details

Weitere Antikörper gegen GDP-Mannose Pyrophosphorylase B Interaktionspartner

Human GDP-Mannose Pyrophosphorylase B (GMPPB) Interaktionspartner

  1. Late-onset limb-girdle muscular dystrophy caused by GMPPB mutations.

  2. Study finds that the GMPPB mutation spectrum in Chinese patients may differ from that of European populations, with the mutation p.(Arg357His) most frequently found. These mutations may lead to abnormal folding of GMPPB leading to protein aggregates in the cytoplasm rather than an overall loss in protein expression.

  3. Patients with GMPPB-CMS have phenotypic features aligned with CMS subtypes harbouring mutations within the early stages of the glycosylation pathway. Additional features shared with the dystroglycanopathies include myopathic features, raised Creatine Kinase levels and variable mild cognitive delay.

  4. We observe that c.79G>C (p.D27H) is associated with a mild limb-girdle muscular dystrophy phenotype, whereas c.860G>A (p.R287Q) is associated with a relatively severe congenital muscular dystrophy typically involving brain development.

  5. This study found mutations in GMPPB can lead to a wide spectrum of clinical features where deficit in neuromuscular transmission is the major component in a subset of cases.

  6. The phenotypic spectrum of GMPPB mutations was expanded to include limb-girdle muscular dystrophies.

  7. Work confirms a role for GMPPB defects in alpha-dystroglycanopathy, and suggests that glycosylation may play a role in the neuronal membrane channels or networks involved in the physiology of generalized epilepsy syndromes.

  8. Individuals with GMPPB mutations have hypoglycosylated alpha-dystroglycan in muscle. These mutations cause congenital and limb-girdle muscular dystrophies.

GDP-Mannose Pyrophosphorylase B (GMPPB) Antigen-Profil

Protein Überblick

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.

Genbezeichner und Symbole assoziert mit GMPPB

  • GDP-mannose pyrophosphorylase B (gmppb) Antikörper
  • GDP-mannose pyrophosphorylase B L homeolog (gmppb.L) Antikörper
  • GDP-mannose pyrophosphorylase B (GMPPB) Antikörper
  • GDP-mannose pyrophosphorylase B (LOAG_13550) Antikörper
  • GDP-mannose pyrophosphorylase B (Gmppb) Antikörper
  • AI317178 Antikörper
  • E430010H19 Antikörper
  • GMPPB Antikörper
  • gmppl Antikörper
  • MDDGA14 Antikörper
  • MDDGB14 Antikörper
  • MDDGC14 Antikörper
  • RGD1560458 Antikörper
  • zgc:92026 Antikörper

Bezeichner auf Proteinebene für GMPPB

GTP-mannose-1-phosphate guanylyltransferase beta , mannose-1-phosphate guanyltransferase beta , GDP-mannose pyrophosphorylase B-A , GTP-mannose-1-phosphate guanylyltransferase beta-A , mannose-1-phosphate guanyltransferase beta-A , GDP-mannose pyrophosphorylase B , mannose-1-phosphate guanyltransferase beta-like

GENE ID SPEZIES
445097 Danio rerio
447346 Xenopus laevis
100856660 Canis lupus familiaris
460378 Pan troglodytes
493267 Xenopus (Silurana) tropicalis
9951021 Loa loa
100028039 Monodelphis domestica
100357646 Oryctolagus cuniculus
100397938 Callithrix jacchus
100436355 Pongo abelii
100474972 Ailuropoda melanoleuca
100583111 Nomascus leucogenys
29925 Homo sapiens
331026 Mus musculus
514161 Bos taurus
100513376 Sus scrofa
363145 Rattus norvegicus
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