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GBA Produits

(Glucosidase, Beta, Acid (GBA))

Catégories

This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2010].

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Catégories GBA en vedette

GBA Anticorps

High quality antibodies with extensive validation data.

GBA Kits ELISA

Reliable ELISA kits for a wide range of species.

GBA Protéines

Proteins for various applications incl. WB, ELISA, IF etc.

GBA recommandé Anticorps

Produit
Reactivity
Application
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Application WB, ELISA, IHC (p), IF
Validations
  • (5)
  • (6)
N° du produit ABIN561009
Quantité 100 μg
Fiche technique Fiche technique
Reactivity Human
Application WB, IHC, IP, ICC
Validations
  • (7)
N° du produit ABIN7434968
Quantité 100 μL
Fiche technique Fiche technique
Reactivity Mouse, Rat
Application IHC, IF
Validations
  • (5)
N° du produit ABIN7074062
Quantité 100 μL
Fiche technique Fiche technique

GBA recommandé Kits ELISA

Produit
Reactivity
Analytical Method
Validations
N° du produit
Quantité
Fiche technique
Reactivity Mouse
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
N° du produit ABIN6967044
Quantité 96 tests
Fiche technique Fiche technique
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
N° du produit ABIN6999571
Quantité 96 tests
Fiche technique Fiche technique
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
N° du produit ABIN6956132
Quantité 96 tests
Fiche technique Fiche technique

GBA recommandé Protéines

Produit
Reactivity
Source
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Source Escherichia coli (E. coli)
Validations
  • (1)
N° du produit ABIN7123107
Quantité 50 μg
Fiche technique Fiche technique
Reactivity Human
Source Wheat germ
Validations
  • (1)
N° du produit ABIN1354861
Quantité 10 μg
Fiche technique Fiche technique
Reactivity Human
Source Wheat germ
Validations
  • (1)
N° du produit ABIN1354863
Quantité 10 μg
Fiche technique Fiche technique

Dernières publications sur nos produits GBA

Bieri, Brahic, Bousset, Couthouis, Kramer, Ma, Nakayama, Monbureau, Defensor, Schüle, Shamloo, Melki, Gitler: "LRRK2 modifies α-syn pathology and spread in mouse models and human neurons." dans: Acta neuropathologica, Vol. 137, Issue 6, pp. 961-980, (2019) (PubMed).

Massaro, Mattar, Wong, Sirka, Buckley, Herbert, Karlsson, Perocheau, Burke, Heales, Richard-Londt, Brandner, Huebecker, Priestman, Platt, Mills, Biswas, Cooper, Chan, Cheng, Waddington, Rahim: "Fetal gene therapy for neurodegenerative disease of infants." dans: Nature medicine, Vol. 24, Issue 9, pp. 1317-1323, (2018) (PubMed).

Zancan, Bellesso, Costa, Salvalaio, Stroppiano, Hammond, Argenton, Filocamo, Moro: "Glucocerebrosidase deficiency in zebrafish affects primary bone ossification through increased oxidative stress and reduced Wnt/β-catenin signaling." dans: Human molecular genetics, Vol. 24, Issue 5, pp. 1280-94, (2015) (PubMed).

Murphy, Gysbers, Abbott, Tayebi, Kim, Sidransky, Cooper, Garner, Halliday: "Reduced glucocerebrosidase is associated with increased ?-synuclein in sporadic Parkinson's disease." dans: Brain : a journal of neurology, Vol. 137, Issue Pt 3, pp. 834-48, (2014) (PubMed).

Bendikov-Bar, Rapaport, Larisch, Horowitz: "Parkin-mediated ubiquitination of mutant glucocerebrosidase leads to competition with its substrates PARIS and ARTS." dans: Orphanet journal of rare diseases, Vol. 9, pp. 86, (2014) (PubMed).

Shu, Vivekanandan-Giri, Pennathur, Smid, Aerts, Hollak, Shayman: "Establishing 3-nitrotyrosine as a biomarker for the vasculopathy of Fabry disease." dans: Kidney international, Vol. 86, Issue 1, pp. 58-66, (2014) (PubMed).

Bendikov-Bar, Horowitz: "Gaucher disease paradigm: from ERAD to comorbidity." dans: Human mutation, Vol. 33, Issue 10, pp. 1398-407, (2012) (PubMed).

Bendikov-Bar, Ron, Filocamo, Horowitz: "Characterization of the ERAD process of the L444P mutant glucocerebrosidase variant." dans: Blood cells, molecules & diseases, Vol. 46, Issue 1, pp. 4-10, (2011) (PubMed).

Campeau, Rafei, Boivin, Sun, Grabowski, Galipeau: "Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome." dans: Blood, Vol. 114, Issue 15, pp. 3181-90, (2009) (PubMed).

Synonymes et noms alternatifs relatifs à GBA

glucosylceramidase beta (GBA), beta glucosidase 25 (BGLU25), beta-glucosidase (PSPTO_3318), beta-glucosidase (bglX), Beta-glucosidase (bglA4), glucosidase, beta, acid (Gba), glucosylceramidase beta (Gba), glucosylceramidase (LOC100399524), beta-glucosidase (bglA.2), bglX-2 (bglX-2), glucosidase, beta, acid (GBA), BETA-GLUCOSIDASE, betaGC, beta glucosidase 25, GBA1, GC, GCase, GCB, GLUC, PSPTO3318, PSPTO4290, T12J13.8, T12J13_8

Protein level used designations for GBA

  • D-glucosyl-N-acylsphingosine glucohydrolase
  • acid beta-glucosidase
  • alglucerase
  • beta-glucocerebrosidase
  • glucosylceramidase
  • imiglucerase
  • lysosomal glucocerebrosidase
  • beta-glucosidase
  • Beta-glucosidase
  • acid beta glucosidase
  • glucocerebrosidase
  • glucosidase, beta; acid
  • glucosidase, beta, acid
  • glucosylceramidase-like
  • glucosidase, beta; acid (includes glucosylceramidase)
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