Anti-GALC Antibody (CAB3873)
- SKU:
- CAB3873
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Metabolism
Frequently bought together:
Description
| Antibody Name: | Anti-GALC Antibody |
| Antibody SKU: | CAB3873 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 40-300 of human GALC (NP_000144.2). |
| Application: | WB IHC |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:100 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | DU145, 293T, HepG2, A375, Mouse brain, Rat testis |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 40-300 of human GALC (NP_000144.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | GGAY VLDD SDGL GREF DGIG AVSG GGAT SRLL VNYP EPYR SQIL DYLF KPNF GASL HILK VEIG GDGQ TTDG TEPS HMHY ALDE NYFR GYEW WLMK EAKK RNPN ITLI GLPW SFPG WLGK GFDW PYVN LQLT AYYV VTWI VGAK RYHD LDID YIGI WNER SYNA NYIK ILRK MLNY QGLQ RVKI IASD NLWE SISA SMLL DAEL FKVV DVIG AHYP GTHS AKDA KLTG KKLW SSED FSTL NSDM GAGC WGRI LNQN YING Y |
| Gene ID: | 2581 |
| Uniprot: | P54803 |
| Cellular Location: | Lysosome |
| Calculated MW: | 67kDa/74kDa/77kDa |
| Observed MW: | 77kDa |
| Synonyms: | GALC |
| Background: | 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. |
| UniProt Protein Function: | GALC: Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. 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. Defects in GALC are the cause of leukodystrophy globoid cell (GLD); also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present 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. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified. Belongs to the glycosyl hydrolase 59 family. 4 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Lipid Metabolism - sphingolipid; EC 3.2.1.46; Hydrolase Chromosomal Location of Human Ortholog: 14q31 Cellular Component: lysosomal lumen; lysosome Molecular Function:galactosylceramidase activity Biological Process: galactosylceramide catabolic process; glycosphingolipid metabolic process Disease: Krabbe Disease |
| NCBI Summary: | 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] |
| UniProt Code: | P54803 |
| NCBI GenInfo Identifier: | 1035664365 |
| NCBI Gene ID: | 2581 |
| NCBI Accession: | P54803.3 |
| UniProt Secondary Accession: | P54803,Q8J030, B4DKE8, B4DYN1, B4DZJ8, B7Z7Z2, J3KN25 J3KPP8, |
| UniProt Related Accession: | P54803 |
| Molecular Weight: | 67,184 Da |
| NCBI Full Name: | Galactocerebrosidase |
| NCBI Synonym Full Names: | galactosylceramidase |
| NCBI Official Symbol: | GALC |
| NCBI Protein Information: | galactocerebrosidase |
| UniProt Protein Name: | Galactocerebrosidase |
| UniProt Synonym Protein Names: | Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidase |
| Protein Family: | Galactocerebrosidase |
| UniProt Gene Name: | GALC |
| UniProt Entry Name: | GALC_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GALC antibody (CAB3873) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
 | Immunohistochemistry of paraffin-embedded human liver cancer using GALC antibody (CAB3873) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse testis using GALC antibody (CAB3873) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse kidney using GALC antibody (CAB3873) at dilution of 1:100 (40x lens). |
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