Collagen XI α1 (Cleaved-Ala1563) rabbit pAb

产品详情

产品名称

Collagen XI α1 (Cleaved-Ala1563) rabbit pAb

产品货号

YM-A03986

规格

50μL;100μL

种属反应

Human,Mouse

实验应用

WB,ELISA

分子量

165kD,200kD

宿主

Rabbit

同种型

IgG

修饰

Unmodified

推荐稀释比

WB 1:1000-2000;ELISA 1:5000-20000

组成

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

纯化工艺

The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.

储存

-15°C to -25°C/1 year(Do not lower than -25°C)

浓度

1 mg/ml

克降性

Polyclonal

克隆号

免疫原

Synthesized peptide derived from human Collagen XI α1 (Cleaved-Ala1563)

特异性

This antibody detects endogenous levels of Human,Mouse Collagen XI α1 (Cleaved-Ala1563, protein was cleaved amino acid sequence between 1563-1564 )

基因名称

COL11A1 COLL6

蛋白名称

Collagen XI α1 (Cleaved-Ala1563)

别名

Collagen alpha-1;XI;chain

基因ID-1

1301

SwissProt

P12107

背景

alternative products:Additional isoforms seem to exist. There is alternative usage of exon IIA or exon IIB. Transcripts containing exon IIA or IIB are present in cartilage, but exon IIB is preferentially utilized in transcripts from tendon,disease:Defects in COL11A1 are the cause of Marshall syndrome [MIM:154780]. It is an autosomal dominant disorder with ocular, orofacial, auditory and skeletal manifestations. It shares several features with Stickler syndrome, such as midfacial hypoplasia, high myopia, and sensorineural-hearing deficit.,disease:Defects in COL11A1 are the cause of Stickler syndrome type 2 (STL2) [MIM:604841]; also known as Stickler syndrome vitreous type 2. STL2 is an autosomal dominant form of Stickler syndrome, an inherited disorder that associates ocular signs with more or less complete forms of Pierre Robin sequence, bone disorders and sensorineural deafness. Ocular disorders may include juvenile cataract, myopia, strabismus, vitreoretinal or chorioretinal degeneration, retinal detachment, and chronic uveitis. Robin sequence includes an opening in the roof of the mouth (a cleft palate), a large tongue (macroglossia), and a small lower jaw (micrognathia). Bones are affected by slight platyspondylisis and large, often defective epiphyses. Juvenile joint laxity is followed by early signs of arthrosis. The degree of hearing loss varies among affected individuals and may become more severe over time. Syndrome expressivity is variable.,function:May play an important role in fibrillogenesis by controlling lateral growth of collagen II fibrils.,PTM:Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,similarity:Belongs to the fibrillar collagen family.,similarity:Contains 1 TSP N-terminal (TSPN) domain.,subunit:Trimers composed of three different chains: alpha 1(XI), alpha 2(XI), and alpha 3(XI). Alpha 3(XI) is a post-translational modification of alpha 1(II). Alpha 1(V) can also be found instead of alpha 3(XI)=1(II).,tissue specificity:Cartilage, placenta and some tumor or virally transformed cell lines. Isoforms using exon IIA or IIB are found in the cartilage while isoforms using only exon IIB are found in the tendon.,

细胞定位

Secreted, extracellular space, extracellular matrix .