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产品信息
PA5-116952
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
A synthetic peptide corresponding to a sequence within amino acids 467-746 of human EXT1 (NP_0001182)
偶联物
形式
Liquid
浓度
1.45 mg/mL
纯化类型
Affinity chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.05% ProClin 300
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
AB_2901582
产品详细信息
Positive Samples: K-562, SW480, PC-3, Mouse liver, Mouse lung
Immunogen sequence: YYYANLGLKP PSKFTAVIHA VTPLVSQSQP VLKLLVAAAK SQYCAQIIVL WNCDKPLPAK HRWPATAVPV VVIEGESKVM SSRFLPYDNI ITDAVLSLDE DTVLSTTEVD FAFTVWQSFP ERIVGYPARS HFWDNSKERW GYTSKWTNDY SMVLTGAAIY HKYYHYLYSH YLPASLKNMV DQLANCEDIL MNFLVSAVTK LPPIKVTQKK QYKETMMGQT SRASRWADPD HFAQRQSCMN TFASWFGYMP LIHSQMRLDP VLFKDQVSIL RKKYRDIERL
靶标信息
Hereditary multiple exostoses (HME) is an autosomal dominant disorder characterized by the formation of exostoses (EXT), which are cartilage-capped bony protuberances mainly located on long bones. Two proteins associated with EXT, EXT1 and EXT2, form homo/heteromeric complexes in vivo, which leads to the accumulation of both proteins in the Golgi apparatus. EXT1 and EXT2 are endoplasmic reticulum-localized type II transmembrane glycoproteins that possess, or are tightly associated with, glycosyltransferase activities involved in the polymerization of the glycosaminoglycan, heparan sulfate (HS). EXT2 is a protein that harbors the D-glucuronyl (GlcA) and N-acetyl-D-glucosaminyl (GlcNAc) transferase activities required for biosynthesis of HS. EXT1 rescues defective HS biosynthesis and elevates low GlcA and GlcNAc transferase levels in mutated cells.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: exostoses (multiple) 1; exostosin 1; Exostosin glycosyltransferase 1; Exostosin-1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Heparan sulfate co-polymerase subunit EXT1; Langer-Giedion syndrome chromosome region; Multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; putative tumor suppressor protein EXT1
基因别名: AA409028; EXT; EXT1; LGCR; LGS; TRPS2; TTV
UniProt ID: (Human) Q16394, (Mouse) P97464
Entrez Gene ID: (Human) 2131, (Rat) 299907, (Mouse) 14042
acetylglucosaminyltransferase activity
glucuronosyltransferase activity
transferase activity, transferring glycosyl groups
heparan sulfate N-acetylglucosaminyltransferase activity
protein homodimerization activity
metal ion binding
protein heterodimerization activity
glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity
molecular_function
transferase activity
glycosyltransferase
skeletal system development
ossification
glycosaminoglycan biosynthetic process
protein glycosylation
signal transduction
gastrulation
axon guidance
endoderm development
mesoderm development
heparan sulfate proteoglycan biosynthetic process
heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process
olfactory bulb development
cellular polysaccharide biosynthetic process
embryonic skeletal joint development
brain development
biological_process
