Galactosidase, alpha Proteine (GLA)

GLA encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. Zusätzlich bieten wir Ihnen GLA Antikörper (127) und GLA Kits (38) und viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
GLA 2717 P06280
GLA 11605  
Ratte GLA GLA 363494  
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Showing 10 out of 18 products:

Katalog Nr. Origin Quelle Konjugat Bilder Menge Anbieter Lieferzeit Preis Details
Insektenzellen Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
Human Cells Maus His tag 50 μg Anmelden zum Anzeigen 20 bis 21 Tage
Human Cells Human His tag 50 μg Anmelden zum Anzeigen 20 bis 21 Tage
Escherichia coli (E. coli) Maus His tag,T7 tag 100 μg Anmelden zum Anzeigen 15 bis 18 Tage
Mammalian Cells Human His tag   50 μg Anmelden zum Anzeigen 4 Days
Wheat germ Human GST tag   10 μg Anmelden zum Anzeigen 11 bis 12 Tage
HEK-293 Cells Human His tag Validation with Western Blot 10 μg Anmelden zum Anzeigen 12 bis 15 Tage
Insektenzellen Human His tag   50 μg Anmelden zum Anzeigen 2 bis 3 Tage
Human Human His tag   10 μg Anmelden zum Anzeigen 15 bis 16 Tage

GLA Proteine nach Spezies und Herkunft

Origin Exprimiert in Konjugat
Human , , , , , ,
, ,
Mouse (Murine) , ,

Am meisten referenzierte GLA Proteine

  1. Human GLA Protein expressed in Wheat germ - ABIN1355176 : Corchero, Mendoza, Lorenzo, Rodríguez-Sureda, Domínguez, Vázquez, Ferrer-Miralles, Villaverde: Integrated approach to produce a recombinant, His-tagged human ?-galactosidase A in mammalian cells. in Biotechnology progress 2011 (PubMed)

  2. Human GLA Protein expressed in Human Cells - ABIN2005026 : Koide, Ishiura, Iwai, Inoue, Kaneda, Okada, Uchida: A case of Fabry's disease in a patient with no alpha-galactosidase A activity caused by a single amino acid substitution of Pro-40 by Ser. in FEBS letters 1990 (PubMed)
    Show all 5 Pubmed References

Weitere Proteine zu Galactosidase, alpha (GLA) Interaktionspartnern

Human Galactosidase, alpha (GLA) Interaktionspartner

  1. Results showed that most Fabry disease patients carrying GLA (zeige NAT8 Proteine) IVS4+919A did not show abnormal cardiac phenotypes. The near-absence of GLA (zeige NAT8 Proteine) IVS4+919A in heart disease cohort suggested that this variant is not a frequent cause of overt heart diseases in Taiwan.

  2. Enzyme activities (acid alpha-glucosidase (GAA), galactocerebrosidase (GALC (zeige GALC Proteine)), glucocerebrosidase (GBA (zeige GBA Proteine)), alpha-galactosidase A (GLA), alpha-iduronidase (IDUA (zeige IDUA Proteine)) and sphingomyeline phosphodiesterase-1 (SMPD-1 (zeige SMPD1 Proteine))) were measured on ~43,000 de-identified dried blood spot (DBS (zeige MCF2L Proteine)) punches, and screen positive samples were submitted for DNA sequencing to obtain genotype confirmation of disease risk

  3. This longitudinal Fabry Registry study analyzed data from patients with Fabry disease to determine the incidence and type of severe clinical events following initiation of enzyme replacement therapy (ERT (zeige ELF3 Proteine)) with agalsidase beta, as well as risk factors associated with occurrence of these events

  4. alpha-galactosidase A mutation, IVS4-type Fabry disease has features similar to those of classic Fabry disease and a higher frequency of deep white matter hyperintensities and a higher incidence of infarctions and pulvinar signs than in healthy controls

  5. We demonstrate that the wild-type sequence harbors an hnRNP A1 (zeige HNRNPA1 Proteine) and hnRNP A2/B1 (zeige HNRNPA2B1 Proteine)-binding exonic splicing silencer (ESS) overlapping the 5'splice site (5'ss) that prevents pseudoexon inclusion.we demonstrate that splice switching oligonucleotide (SSO) mediated blocking of the pseudoexon 3'ss and 5'ss effectively restores normal GLA (zeige NAT8 Proteine) splicing

  6. Four patients had non-amenable mutant forms of a-Gal (zeige GAL Proteine) based on the validated cell-based assay conducted after treatment initiation and were excluded from primary efficacy analyses only.

  7. Mesenchymal stem cells with reduced GLA (zeige NAT8 Proteine) activity are prone to apoptosis and senescence due to impaired autophagy and DNA repair capacity.

  8. we review the various types of GLA (zeige NAT8 Proteine) variants and recommend that pathogenicity be considered only when associated with elevated globotriaosylceramide in disease-relevant organs and tissues as analyzed by mass spectrometry.

  9. findings revealed the alternative splicing mechanism of GLA (zeige NAT8 Proteine) (IVS4+919G>A), and a potential treatment for this specific genetic type of Fabry disease by amiloride in the future

  10. Results found a novel heterozygous stop codon mutation in exon 1 of the GLA (zeige NAT8 Proteine) gene in female patients with Fabry Disease with methylation in the non-mutated allele thought to be associated with the clinical severity of the disease.

Mouse (Murine) Galactosidase, alpha (GLA) Interaktionspartner

  1. Therefore, we confirmed the associations among Golgi function, fibrosis, and autophagy. Moreover, GOLGA2 (zeige GOLGA2 Proteine) knockout mice may be a potentially valuable animal model for studying autophagy-induced fibrosis.

  2. the GM130 (zeige GOLGA2 Proteine)-deficient mouse provides a valuable model for investigating the etiology of human globozoospermia.

  3. Mice with alpha-galactosidase A deficiency show age-dependent and distinct deficits of the sensory system.

  4. In oocyte meiosis, GM130 (zeige GOLGA2 Proteine) localization and expression patterns are regulated by FMNL1 (zeige FMNL1 Proteine).

  5. The histological changes in Gla KO mice better resemble the type 2 later-onset phenotype observed in patients with residual alpha-galactosidase A activity.

  6. our findings imply that the alpha-GalA KO mouse is a good model in which to study the peripheral small fiber neuropathy exhibited by FD patients

  7. we demonstrate an age-dependent microvasculopathy of the mesenteric artery in a murine model of Fabry disease (galactosidase A-knockout mice) resulting from dysregulation of the vascular homeostatic enzyme endothelial nitric oxide synthase (eNOS (zeige NOS3 Proteine))

  8. GM130 (zeige GOLGA2 Proteine) regulates microtubule organization and might cooperate with the MAPK (zeige MAPK1 Proteine) pathway to play roles in spindle organization, migration and asymmetric division during mouse oocyte maturation

  9. It suggested that there could be a combination of GLA deficiency and FVL (zeige F5 Proteine) or other thrombosis-related gene defect in patients with genetic severe early-onset thrombosis.

  10. present Toll (zeige TLR4 Proteine)-like receptor-dependent negative regulation of alpha-Gal-A as a mechanistic link between pathogen recognition and self lipid antigen induction for natural killer T cells

GLA Protein Überblick

Protein Überblick

This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.

Genbezeichner und Symbole assoziert mit Galactosidase, alpha Proteine (GLA)

  • galactosidase alpha (GLA)
  • galactosidase, alpha (Gla)
  • galactosidase, alpha (gla)
  • galactosidase alpha S homeolog (gla.S)
  • galactosidase alpha (gla)
  • alpha-galactosidase (agaN)
  • aga (aga)
  • alpha-galactosidase (agaL)
  • alpha-galactosidase (SCO0541)
  • alpha-galactosidase (rafA)
  • alpha-galactosidase (melA)
  • alpha-galactosidase (galA)
  • alpha-galactosidase A (ANI_1_2528074)
  • alpha-galactosidase A (ANI_1_1502124)
  • alpha-galactosidase A (AOR_1_390174)
  • alpha-galactosidase A (CpipJ_CPIJ002066)
  • alpha-galactosidase (aga)
  • alpha-galactosidase A (MCYG_00962)
  • alpha-galactosidase A (MCYG_00791)
  • alpha-galactosidase (gla)
  • alpha-galactosidase A (Tsp_02909)
  • alpha-galactosidase A (Tsp_02508)
  • golgi autoantigen, golgin subfamily a, 2 (Golga2)
  • galactosidase alpha (Gla)
  • galactosidase, alpha (GLA)
  • Ags Protein
  • alpha-GAL Protein
  • AO090005000217 Protein
  • AW555139 Protein
  • GALA Protein
  • GM130 Protein
  • MGC130872 Protein
  • mKIAA4150 Protein
  • SCF11.21 Protein
  • SMU.877 Protein
  • zgc:101584 Protein

Bezeichner auf Proteinebene für Galactosidase, alpha Proteine (GLA)

agalsidase alfa , alpha-D-galactosidase A , alpha-D-galactoside galactohydrolase 1 , alpha-gal A , alpha-galactosidase A , melibiase , alpha-D-galactoside galactohydrolase , Alpha-galactosidase A , galactosidase, alpha , alpha-galactosidase A-like , alpha-galactosidase , Alpha-galactosidase , 130 kDa cis-Golgi matrix protein , Golgin subfamily A member 2 , SY11 protein , golgin-95

2717 Homo sapiens
11605 Mus musculus
363494 Rattus norvegicus
422188 Gallus gallus
450083 Danio rerio
465761 Pan troglodytes
480988 Canis lupus familiaris
532742 Bos taurus
703129 Macaca mulatta
734749 Xenopus laevis
100022779 Monodelphis domestica
100060370 Equus caballus
100145767 Xenopus (Silurana) tropicalis
100344610 Oryctolagus cuniculus
100409841 Callithrix jacchus
100480888 Ailuropoda melanoleuca
100595113 Nomascus leucogenys
988766 Clostridium perfringens str. 13
1021683 Bifidobacterium longum NCC2705
1028243 Streptococcus mutans UA159
1095964 Streptomyces coelicolor A3(2)
1174420 Yersinia pestis CO92
1255824 Salmonella enterica subsp. enterica serovar Typhimurium str. LT2
2956399 Yersinia pseudotuberculosis IP 32953
3977399 Lactobacillus salivarius UCC118
4983447 Aspergillus niger CBS 513.88
4987403 Aspergillus niger CBS 513.88
5989256 Aspergillus oryzae RIB40
6032795 Culex quinquefasciatus
7264928 Bifidobacterium animalis subsp. lactis AD011
9223595 Arthroderma otae CBS 113480
9226616 Arthroderma otae CBS 113480
9450395 Brachyspira pilosicoli 95/1000
10899858 Trichinella spiralis
10903123 Trichinella spiralis
99412 Mus musculus
100718405 Cavia porcellus
407057 Sus scrofa
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