Rabbit GALC Polyclonal Antibody | anti-GALC antibody

GALC Antibody (N-Term)

Cat. Num. AAA9217245

• Reactivity: Human; Predicted Reactivity: Mouse
• Applications: Western Blot, ELISA
• Synonyms: GALC; Polyclonal Antibody; GALC Antibody (N-Term); Galactocerebrosidase; GALCERase; 3.2.1.46; Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidase; anti-GALC antibody

• Host: Rabbit
• Reactivity: Human; Predicted Reactivity: Mouse
• Clonality: Polyclonal
• Isotype: Ig
• Specificity: This GALC antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 26-56 amino acids from human GALC.
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
• Sequence Length: 685

• Applicable Applications for anti-GALC antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB:~~1:2000
• Subtitle: Purified Rabbit Polyclonal Antibody (Pab)
• Cellular Location: Lysosome.
• Tissue Location: Detected in urine. Detected in testis, brain and placenta (at protein level). Detected in kidney and liver.
• Antigen Type: Synthesized Peptide
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(All lanes : Anti-GALC Antibody (N-Term) at 1:2000 dilutionLane 1: human brain lysateLane 2: Hela whole cell lysateLane 3: NCI-H1299 whole cell lysateLysates/proteins at 20 ug per lane. SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 77 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)

Related Product Information for anti-GALC antibody

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.

Product Categories/Family for anti-GALC antibody

Crown Antibodies; Primary Antibodies; Cardiovascular; Metabolism; Neuroscience; Signal Transduction

References

Luzi P.,et al.Genomics 26:407-409 (1995). Sakai N.,et al.Biochim. Biophys. Acta 1395:62-67 (1998). Ota T.,et al.Nat. Genet. 36:40-45 (2004). Heilig R.,et al.Nature 421:601-607 (2003). Chen Y.Q.,et al.Hum. Mol. Genet. 2:1841-1845 (1993).

NCBI and Uniprot Product Information

• NCBI GI #: 83281450
• NCBI GeneID: 2581
• NCBI Accession #: NP_000144.2
• NCBI GenBank Nucleotide #: NM_000153.3
• UniProt Accession #: P54803; Q8J030; B4DKE8; B4DYN1; B4DZJ8; B7Z7Z2; J3KN25; J3KPP8
• Molecular Weight: 77033 Da
• NCBI Official Full Name: galactocerebrosidase isoform a
• NCBI Official Synonym Full Names: galactosylceramidase
• NCBI Official Symbol: GALC
• NCBI Protein Information: galactocerebrosidase
• UniProt Protein Name: Galactocerebrosidase
• Protein Family: Galactocerebrosidase
• UniProt Gene Name: GALC
• UniProt Synonym Gene Names: GALCERase
• UniProt Entry Name: GALC_HUMAN

NCBI Description

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 Description

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.

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: carbohydrate metabolic process; galactosylceramide catabolic process; glycosphingolipid metabolic process

Disease: Krabbe Disease