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Invitrogen
EXT1 Polyclonal Antibody
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图: 1 / 6
EXT1 Antibody (PA5-106907) in IHC (P)
Immunohistochemistry analysis of EXT1 in mouse muscle tissue. The sample was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. Samples were incubated with EXT1 polyclonal antibody (Product # PA5-106907) using a dilution of 1:100 (4°C overnight) followed by HRP conjugated anti-Rabbit secondary antibody.
Please note: We are reviewing Western blot images included in the antibody testing data in our catalog, including those provided by third parties. Unless expressly labeled or annotated as “raw-unedited”, Western blot images included in the antibody testing data in our catalog may have been edited, optimized or otherwise adjusted for presentation.
产品信息
PA5-106907
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse,
Rat
已发表种属
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
A synthesized peptide derived from human EXT1(Accession Q16394), corresponding to amino acid residues R6-P56.
偶联物
Unconjugated
Unconjugated
Unconjugated
形式
Liquid
浓度
1 mg/mL
规格
100 µg
纯化类型
Affinity chromatography
保存液
PBS, pH 7.4, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Antibody detects endogenous levels of total EXT1.
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。
生物信息学
蛋白别名: exostoses (multiple) 1; exostosin 1; Exostosin glycosyltransferase 1; Exostosin-1; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Heparan sulfate co-polymerase subunit EXT1; Multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase
基因别名: AA409028; EXT1
UniProt ID: (Mouse) P97464
Entrez Gene ID: (Rat) 299907, (Mouse) 14042
molecular_function
acetylglucosaminyltransferase activity
glucuronosyltransferase activity
transferase activity, transferring glycosyl groups
protein homodimerization activity
protein heterodimerization activity
glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity
transferase activity
metal ion binding
glycosyltransferase
ossification
glycosaminoglycan biosynthetic process
gastrulation
axon guidance
brain development
endoderm development
mesoderm development
biological_process
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
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Clicking the images or links will redirect you to a website hosted by BenchSci that provides third-party scientific content. Neither the content nor the BenchSci technology and processes for selection have been evaluated by us; we are providing them as-is and without warranty of any kind, including for use or application of the Thermo Fisher Scientific products presented.
