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Mouse Anti-GALC Recombinant Antibody (2D1) (CBMAB-G1844-LY)

This product is antibody recognizes GALC. The antibody 2D1 immunoassay techniques such as: ELISA, WB.
See all GALC antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2D1
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
GALC (AAH36518, 27 a.a. ~ 669 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: YVLDDSDGLG REFDGIGAVS GGGATSRLLV NYPEPYRSQI LDYLFKPNFG ASLHILKVEI GGDGQTTDGT EPSHMHYALD GNYFRGYEWW LMKEAKKRNP NITLIGLPWS FPGWLGKGFD WPYVNLQLTA YYVVTWIVGA KRYHDLDIDY IGIWNERSYN ANYIKILRKM LNYQGLQRVK IIASDNLWES ISASMLLDAE LFKVVDVIGA HYPGTHSAKD AKLTGKKLWS SEDFSTLNSD MGAGCWGRIL NQNYINGYMT STIAWNLVAS YYEQLPYGRC GLMTAQEPWS GHYVVESPVW VSAHTTQFTQ PGWYYLKTVG HLEKGGSYVA LTDGLGNLTI IIETMSHKHS KCIRPFLPYF NVSQQFATFV LKGSFSEIPE LQVWYTKLGK TSERFLFKQL DSLWLLDSDG SFTLSLHEDE LFTLTTLTTG RKGSYPLPPK SQPFPSTYKD DFNVDYPFFS EAPNFADQTG VFEYFTNIED PGEHHFTLRQ VLNQRPITWA ADASNTISII GDYNWTNLTT KCDVYIETPD TGGVFIAGRV NKGGILIRSA RGIFFWIFAN GSYRVTGDLA GWIIYALGRV EVTAKKWYTL TLTIKGHFAS GMLNDKSLWT DIPVNFPKNG WAAIGTHSFE FAQFDNFRVE ATR
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
galactosylceramidase
Introduction
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. [provided by RefSeq, Jul 2008]
Entrez Gene ID
2581
UniProt ID
P54803
Alternative Names
Galactosylceramidase; Galactocerebroside Beta-Galactosidase; Galactosylceramide Beta-Galactosidase; EC 3.2.1.46; GALCERase; Testis Tissue Sperm-Binding Protein Li 88E;
Function
Hydrolyzes the galactose ester bonds of glycolipids such as galactosylceramide and galactosylsphingosine (PubMed:8281145, PubMed:8399327).

Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon (PubMed:8281145, PubMed:8399327).
Biological Process
Galactosylceramide catabolic process Source: UniProtKB
Glycosphingolipid metabolic process Source: Reactome
Myelination Source: Ensembl
Cellular Location
Lysosome
Involvement in disease
Leukodystrophy, globoid cell (GLD):
Disease descriptionAn autosomal recessive disorder characterized by insufficient catabolism of several galactolipids that are important for normal myelin production. Four clinical forms are recognized. The infantile form accounts for 90% of cases. It manifests before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cerebral hyperpyrexia before 2 years of age. Cases with later onset present with unexplained blindness, weakness and sensorimotor peripheral neuropathy, mental deterioration and death.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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