anti-GDP-Mannose Pyrophosphorylase B (GMPPB) Anticorps

GMPPB is thought to encode a GDP-mannose pyrophosphorylase. De plus, nous expédions GDP-Mannose Pyrophosphorylase B Protéines (7) et et beaucoup plus de produits pour cette protéine.

afficher tous les anticorps Gène GeneID UniProt
GMPPB 29925 Q9Y5P6
GMPPB 331026 Q8BTZ7
GMPPB 363145  
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Top anti-GDP-Mannose Pyrophosphorylase B Anticorps sur anticorps-enligne.fr

Showing 10 out of 45 products:

Catalogue No. Reactivité Hôte Conjugué Application Images Quantité Fournisseur Livraison Prix Détails
Boeuf (Vache) Lapin Inconjugué WB WB Suggested Anti-Gmppb Antibody Titration: 1.0 ug/ml Positive Control: Mouse Pancreas 100 μL Connectez-vous pour afficher 2 to 3 Days
$289.00
Détails
Boeuf (Vache) Lapin Inconjugué IHC, WB Human kidney 100 μL Connectez-vous pour afficher 2 to 3 Days
$229.00
Détails
Boeuf (Vache) Lapin Inconjugué IHC, IHC (p), WB 100 μg Connectez-vous pour afficher 7 to 9 Days
$493.17
Détails
Fruit Fly Lapin Inconjugué WB 50 μg Connectez-vous pour afficher 7 to 9 Days
$551.83
Détails
Humain Souris Inconjugué ELISA, IHC, IHC (p), WB 100 μg Connectez-vous pour afficher 7 to 9 Days
$537.17
Détails
Humain Souris Inconjugué 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 Connectez-vous pour afficher 11 to 12 Days
$440.00
Détails
Humain Souris Inconjugué ELISA, IHC, WB GMPPB monoclonal antibody (M07), clone 2B5 Western Blot analysis of GMPPB expression in HepG2 on formalin-fixed paraffin-embedded human adrenal gland (antibody concentration 3 µg/mL). 100 μg Connectez-vous pour afficher 8 to 11 Days
$461.00
Détails
Humain Lapin Inconjugué 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 Connectez-vous pour afficher 2 to 3 Days
$515.63
Détails
Souris Lapin Inconjugué WB Western Blot at 1:4000 dilution + mouse pancreas lysate Lysates/proteins at 20 ug per lane. 200 μL Connectez-vous pour afficher 2 to 3 Days
$515.63
Détails
Humain Lapin Inconjugué IHC, IHC (p), WB Western Blot: GMPPB Antibody [NBP1-55168] - HepG2 cell lysate, concentration 5.0ug/ml. Immunohistochemistry-Paraffin: GMPPB Antibody [NBP1-55168] - Human kidney Tissue, antibody concentration 4-8ug/ml. Cells with positive label: renal corpuscle cells (indicated with arrows) 400X magnification. 100 μL Connectez-vous pour afficher 8 to 11 Days
$444.19
Détails

GMPPB Anticorps par réactivité, application, clonalité et conjugué

Attributs Application Hôte Clonalité Conjugué
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Plus d’anticorps contre GDP-Mannose Pyrophosphorylase B partenaires d’interaction

Human GDP-Mannose Pyrophosphorylase B (GMPPB) interaction partners

  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 (Montrer Cd2ap Anticorps) have phenotypic features aligned with CMS (Montrer Cd2ap Anticorps) 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. 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.

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

  6. 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.

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

GDP-Mannose Pyrophosphorylase B (GMPPB) profil antigène

Profil protéine

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.

Gene names and symbols associated with GMPPB

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

Protein level used designations for 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 SPECIES
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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