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GALNS Rabbit pAb (bs-13269R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價
產(chǎn)品編號 bs-13269R
英文名稱 GALNS Rabbit pAb
中文名稱 硫酸軟骨素裂解酶抗體
別    名 Chondroitinase; Chondroitinsulfatase; FLJ00319; FLJ17434; FLJ42844; FLJ98217; Galactosamine(N acetyl) 6 sulfate sulfatase; Galactose 6 sulfate sulfatase; GALNAC6S; GalNAc6S sulfatase; GAS; mFLJ00319; MPS4A; N acetylgalactosamine 6 sulfatase; N acetylgalac  
研究領(lǐng)域 細(xì)胞生物  信號轉(zhuǎn)導(dǎo)  泛素  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human,Mouse,Rat,Pig,Sheep,Cow,Dog,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 55 kDa
檢測分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GALNS: 1-100/522 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 Chondroitinase is a 522 amino acid protein that localizes to the lysosome and functions as an exohydrolase that is essential for the degradation of glycosaminoglycans, keratan sulfate and chondroitin 6-sulfate. Using calcium as a cofactor, Chondroitinase, which exists as a disulfide linked oligomer, catalyzes the hydrolysis of the 6-sulfate group on target substrates. Defects in the gene encoding Chondroitinase are the cause of mucopolysaccharidosis type 4A (MPS4A), an autosomal recessive lysosomal storage disease that is characterized by the intracellular accumulation of keratan sulfate and chondroitin-6-sulfate and is associated with dental anomalies, short stature and, in some cases, death in the second or third decade of life.

Function:
GALNS (N-acetylgalactosamine-6-sulfatase) is a lysosomal exohydrolase which catalyzes the hydrolysis of the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate and of the D-galactose 6-sulfate units of keratan sulfate. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder.

Subunit:
Oligomer of disulfide linked 40- and 15 kDa polypeptides.

Subcellular Location:
Lysosome.

Post-translational modifications:
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity (By similarity).

DISEASE:
Defects in GALNS are the cause of mucopolysaccharidosis type 4A (MPS4A) [MIM:253000]; 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.

Similarity:
Belongs to the sulfatase family.

SWISS:
P34059

Gene ID:
2588

Database links:

Entrez Gene: 2588 Human

Entrez Gene: 50917 Mouse

Entrez Gene: 292073 Rat

Omim: 612222 Human

SwissProt: P34059 Human

SwissProt: Q571E4 Mouse

SwissProt: Q32KJ6 Rat



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