Cell Biology Antibodies 9

Anti-GALNS Antibody (CAB5461)

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SKU:
CAB5461
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-GALNS Antibody
Antibody SKU:CAB5461
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 353-522 of human GALNS (NP_000503.1).
Application:WB IF
Recommended Dilution:WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:MCF7, HT-29, 293T, Mouse kidney, Mouse brain, Mouse intestine
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 353-522 of human GALNS (NP_000503.1).
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:AGLT PPSD RAID GLNL LPTL LQGR LMDR PIFY YRGD TLMA ATLG QHKA HFWT WTNS WENF RQGI DFCP GQNV SGVT THNL EDHT KLPL IFHL GRDP GERF PLSF ASAE YQEA LSRI TSVV QQHQ EALV PAQP QLNV CNWA VMNW APPG CEKL GKCL TPPE SIPK KCLW SH
Gene ID:2588
Uniprot:P34059
Cellular Location:Lysosome
Calculated MW:58kDa
Observed MW:55kDa
Synonyms:GALNS, GALNAC6S, GAS, GalN6S, MPS4A
Background:This gene encodes N-acetylgalactosamine-6-sulfatase which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate. Sequence alterations including point, missense and nonsense mutations, as well as those that affect splicing, result in a deficiency of this enzyme. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder.
UniProt Protein Function:GALNS: Defects in GALNS are the cause of mucopolysaccharidosis type 4A (MPS4A); also known as Morquio A syndrome. MPS4A is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life. Belongs to the sulfatase family.
UniProt Protein Details:

Protein type:Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.1.6.4

Chromosomal Location of Human Ortholog: 16q24.3

Cellular Component: lysosomal lumen

Molecular Function:N-acetylgalactosamine-4-sulfatase activity; N-acetylgalactosamine-6-sulfatase activity; sulfuric ester hydrolase activity; metal ion binding

Biological Process: keratan sulfate metabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; keratan sulfate catabolic process

Disease: Mucopolysaccharidosis, Type Iva

NCBI Summary:This gene encodes N-acetylgalactosamine-6-sulfatase which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate. Sequence alterations including point, missense and nonsense mutations, as well as those that affect splicing, result in a deficiency of this enzyme. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder. [provided by RefSeq, Jul 2008]
UniProt Code:P34059
NCBI GenInfo Identifier:462148
NCBI Gene ID:2588
NCBI Accession:P34059.1
UniProt Secondary Accession:P34059,Q86VK3,
UniProt Related Accession:P34059
Molecular Weight:522
NCBI Full Name:N-acetylgalactosamine-6-sulfatase
NCBI Synonym Full Names:galactosamine (N-acetyl)-6-sulfatase
NCBI Official Symbol:GALNS  
NCBI Official Synonym Symbols:GAS; MPS4A; GalN6S; GALNAC6S  
NCBI Protein Information:N-acetylgalactosamine-6-sulfatase; chondroitinase; galNAc6S sulfatase; chondroitinsulfatase; galactose-6-sulfate sulfatase; N-acetylgalactosamine-6-sulfate sulfatase; galactosamine (N-acetyl)-6-sulfate sulfatase
UniProt Protein Name:N-acetylgalactosamine-6-sulfatase
UniProt Synonym Protein Names:Chondroitinsulfatase; Chondroitinase; Galactose-6-sulfate sulfatase; GalN6S; N-acetylgalactosamine-6-sulfate sulfatase; GalNAc6S sulfatase
Protein Family:N-acetylgalactosamine-6-sulfatase
UniProt Gene Name:GALNS  
UniProt Entry Name:GALNS_HUMAN
Anti-GALNS Antibody (CAB5461)Western blot analysis of extracts of various cell lines, using GALNS antibody (CAB5461) 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: 15s.
Anti-GALNS Antibody (CAB5461)Immunofluorescence analysis of C6 cells using GALNS Rabbit pAb (CAB5461) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
Anti-GALNS Antibody (CAB5461)Immunofluorescence analysis of L929 cells using GALNS Rabbit pAb (CAB5461) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
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