anti-Galactosylceramidase (GALC) Antikörper

GALC encodes a lysosomal protein which hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Zusätzlich bieten wir Ihnen Galactosylceramidase Kits (10) und Galactosylceramidase Proteine (10) und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
GALC 2581 P54803
GALC 14420 P54818
GALC 314360  
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Top anti-Galactosylceramidase Antikörper auf antikoerper-online.de

Showing 10 out of 76 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Rind (Kuh) Kaninchen Unkonjugiert WB WB Suggested Anti-GALC Antibody Titration:  0.2-1 ug/ml  ELISA Titer:  1:62500  Positive Control:  Jurkat cell lysate 100 μL Anmelden zum Anzeigen 2 bis 3 Tage
$319.00
Details
Human Kaninchen Unkonjugiert WB WB Image Sample (30 ug of whole cell lysate) A: H1299 7.5% SDS PAGE Galactosylceramidase antibody antibody diluted at 1:1000 100 μL Anmelden zum Anzeigen 3 bis 4 Tage
$466.18
Details
Human Kaninchen Unkonjugiert IF (p), IHC (p), WB Formalin-fixed and paraffin embedded rat brain tissue labeled with Anti-Galc Polyclonal Antibody, Unconjugated (ABIN708101) at 1:200 followed by conjugation to the secondary antibody, (SP-0023), and DAB staining L1 rat brain, L2 rat liver lysates probed (ABIN708101) at 1:200 in 4 °C. Followed by conjugation to secondary antibody at 1:3000 90min in 37 °C. Predicted and observed band size: 71kDa. 100 μL Anmelden zum Anzeigen 3 bis 7 Tage
$317.90
Details
Human Kaninchen Unkonjugiert ICC, IHC, WB Western Blot; Sample: Human Lung lysate; Primary Ab: 2µg/ml Rabbit Anti-Human GALC Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (Catalog: SAA544Rb19) 100 μg Anmelden zum Anzeigen 15 bis 18 Tage
$350.00
Details
Fledermaus Kaninchen Unkonjugiert WB 50 μg Anmelden zum Anzeigen 11 bis 14 Tage
$551.83
Details
Human Kaninchen Unkonjugiert IHC, WB ABIN6272899 at 1/100 staining Human brain cancer tissue by IHC-P. The sample was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The sample was then blocked and incubated with the antibody for 1.5 hours at 22°C. An HRP conjugated goat anti-rabbit antibody was used as the secondary. Western blot analysis of extracts of A375 cells, using Galc antibody. 100 μL Anmelden zum Anzeigen 11 bis 12 Tage
$390.77
Details
Human Kaninchen Unkonjugiert IHC, IHC (p), WB Immunohistochemistry-Paraffin: Galactosylceramidase/GALC Antibody [NBP2-16578] - Paraffin-embedded rat liver. Galactosylceramidase antibody diluted at 1:400. Western Blot: GALC Antibody [NBP2-16578] - Sample (30 ug of whole cell lysate) A: H1299 7. 5% SDS PAGE gel, diluted at 1:1000. 0.1 mL Anmelden zum Anzeigen 8 bis 11 Tage
$516.98
Details
Ratte Kaninchen Unkonjugiert ICC, IHC, WB Figure.DAB staining on IHC-P. Samples: Rat Tissue 100 μg Anmelden zum Anzeigen 13 bis 16 Tage
$380.00
Details
Human Kaninchen Unkonjugiert FACS, WB Overlay histogram showing Hela cells stained with Antibody (green line). The cells were fixed with 2% paraformaldehyde (10 min) and then permeabilized with 90% methanol for 10 min. The cells were then icubated in 2% bovine serum albumin to block non-specific protein-protein interactions followed by the antibody (1:25 dilution) for 60 min at 37ºC. The secondary antibody used was Goat-Anti-Rabbit IgG, DyLight 488 Conjugated Highly Cross-Adsorbed(OH191631) at 1/200 dilution for 40 min at 37ºC. Isotype control antibody (blue line) was rabbit IgG (1ug/1x10^6 cells) used under the same conditions. Acquisition of >10, 000 events was performed. Western Blot at 1:2000 dilution Lane 1: human brain lysate Lane 2: Hela whole cell lysate Lane 3: NCI-H1299 whole cell lysate Lysates/proteins at 20 ug per lane. 200 μL Anmelden zum Anzeigen 2 bis 3 Tage
$515.63
Details
Human Kaninchen Unkonjugiert IF/ICC, IHC, IP, WB Western blot analysis of Human Lung lysate, using Human GALC Antibody (2 µg/ml) and HRP-conjugated Goat Anti-Rabbit antibody ( Western blot analysis of the recombinant protein. 100 μg Anmelden zum Anzeigen 11 bis 18 Tage
$326.91
Details

Weitere Antikörper gegen Galactosylceramidase Interaktionspartner

Human Galactosylceramidase (GALC) Interaktionspartner

  1. rs17203398 associated with mucous membrane pemphigoid

  2. The authors present the structure of a glycosphingolipid-processing complex, revealing how SapA and GALC form a heterotetramer with an open channel connecting the enzyme active site to the SapA hydrophobic cavity.

  3. EMT phenotypes and GALC expression of CTCs are correlated with cancer metastasis and therapeutic outcomes, suggesting them to be potential markers for the prognosis of NSCLC.

  4. Results show for the first time that during normal aging, psychosine metabolism is progressively dysfunctional, leading to its accumulation in the aging brain, especially in regions vulnerable to degeneration in Parkinson's disease (PD). These results underline the possibility that defects in the metabolism mediated by changes in GALC activity modify the risk of developing alpha-synuclein pathology in vulnerable patients.

  5. we showed for the first time the specific alteration of beta-Galctosidase (Gal), beta-Galactosylcerebrosidase (GALC) in MCI patients. It is notable that in above peripheral biological samples the lysosomes are more sensitive to AD cellular metabolic alteration when compared to levels of Abeta-peptide or Tau proteins, similar in both AD groups analyzed

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

  7. This review illustrated The Krabbe's disease is caused by mutation in the enzyme Beta-galactocerebrosidase.

  8. This is the largest expression study of GALC variants/mutations found in Newborn screening and confirmed KD cases.

  9. We demonstrated that a heterozygous GALC deletion does not play a significant role in the pathogenesis of normal tension glaucoma in a representative clinic-based population of South Koreans, unlike whites.

  10. Mutations in GALC cause late-onset Krabbe disease with predominant cerebellar ataxia

  11. promoter hypermethylation contributed to down-regulation of GALC Gene, implicating epigenetic inactivation of GALC may play a role in tumorigenesis of cancer.

  12. Case Report: compound GALC heterozygosity in a boy with infantile Krabbe disease with severe clinical course.

  13. GALC was downregulated by promoter hypermethylation and contributed to the pathogenesis of EBV-associated nasopharyngeal carcinoma

  14. Genetic variants near IREB2 and GALC likely contribute to genetic susceptibility to PAE associated with COPD.

  15. Itis an exoglycosidase that catalyzes the hydrolysis of terminal beta-linked galactose residues and its deficiencies or mutation cause llysosomal diseases. (review)

  16. Structural snapshots illustrate the catalytic cycle of beta-galactocerebrosidase, the defective enzyme in Krabbe disease.

  17. Higher galactocerebrosidase activity is predictive of later symptom onset times but does not predict survival after symptom onset when controlling for the logarithm of age at onset.

  18. study identified 4 novel mutations of the GALC gene in 2 unrelated Chinese families with Krabbe disease: one insertion mutation, c.1836_1837insT, one nonsense mutation, c.599C>A (p.S200X),one deletion mutation, c.1911 1_1911 5delGTAAG and one missense mutation, c.2041G>A

  19. GALC mutation is associated with Krabbe disease.

  20. The p.Gly41Ser mutation was associated with longer survival with Krabbe disease. A wide spectrum of late onset Krabbe leukodystrophy is found despite similar genotype.

Mouse (Murine) Galactosylceramidase (GALC) Interaktionspartner

  1. Data show that transgenic galactocerebrosidase (GALC) activity was mainly localized at the Purkinje cells layer in the cerebellum of the AAV-treated twitcher mice.

  2. The results of this study indicated that the greater neurochemical pathology observed in the optic nerve than in the sciatic nerve of beta-gal -/- mice is likely due to the greater glycolipid storage in optic nerve.

  3. This study demonistrated that galactosylceramidase Deficiency also casue neuromuscular dysfunction.

  4. results show that GALCtwi-5J, a spontaneous mutation in murine GALC precisely matches the E130K missense mutation in patients with infantile Krabbe disease

  5. Insights into the mechanisms underlying galactosylceramidase regulation of early post-natal neurogenic niches improve our understanding of the multi-component pathology of globoid cell leukodystrophy .

  6. The crystal structures of GALC and the GALC-product complex, revealing a novel domain architecture with a previously uncharacterized lectin domain not observed in other hydrolases, are presented.

  7. Data show that GALC and, possibly, other enzymes for the maintenance of niche functionality and health tightly control the concentration of these sphingolipids within HSPCs.

  8. GALC is not restricted to myelinating cells but also to several neuronal cell types in the nervous system, such as hippocampal pyramidal neurons and cerebellar neurons.

  9. Direct administration of these viral particles into the brains of neonatal mice with globoid cell leukodystrophy resulted in sustained expression of GALC activity, improved myelination

  10. mutant oligodendrocytes can internalize exogenous galactocerebrosidase and maintain stable myelin, demonstrating that exogenous enzyme replacement will be a key strategy in the therapy of globoid cell leukodystrophy

  11. Lentiviral vectors were designed and optimized for transfer of Galc expression in Twitcher brain.

  12. Mouse model of globoid cell leukodystrophy contains a premature stop codon (W339X) in the galactosylceramidase (GALC) gene that abolishes enzymatic activity.

  13. These results suggest that GALC deficiency not only affects myelinating glia but also leads to neuronal dysfunction. The contemporaneous neuropathology might help to explain the limited efficacy of current gene and cell therapies.

Galactosylceramidase (GALC) Antigen-Profil

Protein Überblick

This gene encodes a lysosomal protein which hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Mutations in this gene have been associated with Krabbe disease, also known as globoid cell leukodystrophy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

Genbezeichner und Symbole assoziert mit GALC

  • galactosylceramidase (GALC) Antikörper
  • galactosylceramidase a (galca) Antikörper
  • galactosylceramidase (galc) Antikörper
  • Galactosylceramidase (Caci_7107) Antikörper
  • Galactosylceramidase (Bacsa_1415) Antikörper
  • Galactosylceramidase (Spico_0384) Antikörper
  • Galactosylceramidase (LOC100304802) Antikörper
  • galactosylceramidase L homeolog (galc.L) Antikörper
  • galactosylceramidase (Galc) Antikörper
  • 2310068B06Rik Antikörper
  • A930008M05Rik Antikörper
  • AW212969 Antikörper
  • AW413532 Antikörper
  • Gacy Antikörper
  • galc Antikörper
  • GALCERase Antikörper
  • galcl Antikörper
  • si:ch211-199l3.4 Antikörper
  • twi Antikörper
  • twitcher Antikörper
  • zgc:92561 Antikörper

Bezeichner auf Proteinebene für GALC

galactocerebrosidase , GALCERase , galactosylceramidase like , Galactosylceramidase , Galactocerebrosidase , galactocerebroside beta-galactosidase , galactosylceramide beta-galactosidase , galactosylceraminidase , galactosylceramidase (Krabbe disease)

GENE ID SPEZIES
100034130 Equus caballus
449649 Danio rerio
779723 Xenopus (Silurana) tropicalis
8338474 Catenulispora acidiphila DSM 44928
10259317 Bacteroides salanitronis DSM 18170
10494670 Sphaerochaeta coccoides DSM 17374
100304802 Ictalurus punctatus
100195231 Salmo salar
446948 Xenopus laevis
2581 Homo sapiens
403916 Canis lupus familiaris
533428 Bos taurus
14420 Mus musculus
314360 Rattus norvegicus
693322 Macaca mulatta
423394 Gallus gallus
100156917 Sus scrofa
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