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Product Name
Galc antibody
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Description
Galc Rabbit Polyclonal antibody. Positive WB detected in mouse brain tissue, A375 cells. Positive IHC detected in human gliomas tissue. Observed molecular weight by Western-blot: 80kd,30kd
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Tested applications
ELISA, WB, IHC
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Species reactivity
Human,Mouse,Rat; other species not tested.
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Alternative names
2310068B06Rik antibody; A930008M05Rik antibody; AW212969 antibody; AW413532 antibody; Gacy antibody; galactosylceramidase antibody; Galc antibody; twi antibody; twitcher antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of Galc recombinant protein (Accession Number: BC086671). Purification method: Antigen affinity purified.
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Clonality
Polyclonal
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Formulation
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
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Storage instructions
Store at -20℃. DO NOT ALIQUOT
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Applications
Recommended Dilution:
WB: 1:500-1:5000
IHC: 1:20-1:200
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Validations
mouse brain tissue were subjected to SDS PAGE followed by western blot with Catalog No:110832(Galc antibody) at dilution of 1:300
Immunohistochemical of paraffin-embedded human gliomas using Catalog No:110832(Galc antibody) at dilution of 1:50 (under 10x lens)
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Background
The GALC antibody targets the liposomal enzyme Galactosylceramidase (GALC), which belongs to the glycosyl hydrolase 59 family. It hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is primarily found in the brain and kidneys where galactolipids are hydrolyzed(PMID:8634707). Deficiencies of GALC are primarily associated with the autosomal recessive Krabbe’s disease. This disease is characterized by developmental delay caused by apoptosis of myelin-forming cells. GALC is responsible for hydrolyzing galactosylceramide, a cerebroside that is an important component of myelin. A deficiency in GALC causes loss of myelin to nerve cells, resulting in delayed nerve transmissions. Krabbe’s disease has varying degrees of severity due to a large number of different genetic mutations in the gene. The GALC antibody can be used to detect the deletions in the GALC gene and functions of the enzyme(PMID:20886637).Normal GALC mRNA encodes the 80 kDa precursor, which is processed into 50 and 30 kDa subunits. (PMID:8634707 )
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References
- Zizioli D, Guarienti M, Tobia C. Molecular cloning and knockdown of galactocerebrosidase in zebrafish: new insights into the pathogenesis of Krabbe's disease. Biochimica et biophysica acta. 1842(4):665-75. 2014.
- Rafi MA, Rao HZ, Luzi P, Luddi A, Curtis MT, Wenger DA. Intravenous injection of AAVrh10-GALC after the neonatal period in twitcher mice results in significant expression in the central and peripheral nervous systems and improvement of clinical features. Molecular genetics and metabolism. 114(3):459-66. 2015.
- Zhao Y, Guo Y, Wang Z. GALC gene is downregulated by promoter hypermethylation in Epstein-Barr virus-associated nasopharyngeal carcinoma. Oncology reports. 34(3):1369-78. 2015.
- Rafi MA, Zhi Rao H, Passini MA. AAV-mediated expression of galactocerebrosidase in brain results in attenuated symptoms and extended life span in murine models of globoid cell leukodystrophy. Molecular therapy : the journal of the American Society of Gene Therapy. 11(5):734-44. 2005.
- Rafi MA, Rao HZ, Luzi P, Curtis MT, Wenger DA. Extended normal life after AAVrh10-mediated gene therapy in the mouse model of Krabbe disease. Molecular therapy : the journal of the American Society of Gene Therapy. 20(11):2031-42. 2012.
- Bashir T, Cloninger C, Artinian N. Conditional astroglial Rictor overexpression induces malignant glioma in mice. PloS one. 7(10):e47741. 2012.
- Scruggs BA, Zhang X, Bowles AC. Multipotent stromal cells alleviate inflammation, neuropathology, and symptoms associated with globoid cell leukodystrophy in the twitcher mouse. Stem cells (Dayton, Ohio). 31(8):1523-34. 2013.
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