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GBA 产品

(Glucosidase, Beta, Acid (GBA))

Categories

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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Featured GBA Categories

GBA 抗体

High quality antibodies with extensive validation data.

GBA ELISA试剂盒

Reliable ELISA kits for a wide range of species.

GBA 蛋白

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

Recommended GBA 抗体

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application WB, ELISA, IHC (p), IF
Validations
  • (5)
  • (6)
Cat. No. ABIN561009
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Application WB, IHC, IP, ICC
Validations
  • (7)
Cat. No. ABIN7434968
Quantity 100 μL
Datasheet Datasheet
Reactivity Mouse, Rat
Application IHC, IF
Validations
  • (5)
Cat. No. ABIN7074062
Quantity 100 μL
Datasheet Datasheet

Recommended GBA ELISA试剂盒

Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity Mouse
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
Cat. No. ABIN6967044
Quantity 96 tests
Datasheet Datasheet
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
Cat. No. ABIN6999571
Quantity 96 tests
Datasheet Datasheet
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
Cat. No. ABIN6956132
Quantity 96 tests
Datasheet Datasheet

Recommended GBA 蛋白

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Source Escherichia coli (E. coli)
Validations
  • (1)
Cat. No. ABIN7123107
Quantity 50 μg
Datasheet Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1354865
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1354861
Quantity 10 μg
Datasheet Datasheet

Latest Publications for our GBA Products

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." in: 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." in: 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." in: 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." in: 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." in: 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." in: Kidney international, Vol. 86, Issue 1, pp. 58-66, (2014) (PubMed).

Bendikov-Bar, Horowitz: "Gaucher disease paradigm: from ERAD to comorbidity." in: 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." in: 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." in: Blood, Vol. 114, Issue 15, pp. 3181-90, (2009) (PubMed).

Synonyms and alternative names related to 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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