Collagen XI alpha 2,胶原蛋白11A2抗体

产品编号HZ-2924R
英文名称Collagen XI alpha 2
中文名称胶原蛋白11A2抗体
别 名Collagen XI alpha 2; COBA2_HUMAN; COL11A2; Collagen alpha 2(XI); Collagen alpha-2(XI) chain; Collagen type XI alpha 2; DAQB-79P13.8; DFNA13; HGNC:2187; HKE5; PARP; STL3; COBA2_HUMAN.
说 明 书0.1ml 0.2ml
研究领域免疫学 神经生物学 信号转导
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Dog, Pig, Horse, Rabbit,
产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量169kDa
Collagen XI alpha 2,胶原蛋白11A2抗体细胞定位细胞外基质 分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Collagen XI alpha 2
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Collagen XI alpha 2,胶原蛋白11A2抗体PubMedPubMed
产品介绍background:
May play an important role in fibrillogenesis by controlling lateral growth of collagen II fibrils.

Function:
May play an important role in fibrillogenesis by controlling lateral growth of collagen II fibrils.

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).

Subcellular Location:
Secreted. extracellular space. extracellular matrix.

Post-translational modifications:
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
A disulfide-bonded peptide called proline/arginine-rich protein or PARP is released from the N-terminus during extracellular processing and is subsequently retained in the cartilage matrix from which it can be isolated in significant amounts.

DISEASE:
Defects in COL11A2 are the cause of Stickler syndrome type 3 (STL3) [MIM:184840]. STL3 is an autosomal dominant non-ocular form of Stickler syndrome. Classical Stickler syndrome associates ocular signs with more or less complete forms of Pierre Robin sequence, bone disorders and sensorineural deafness. Ocular symptoms are absent in STL3. 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.
Collagen XI alpha 2,胶原蛋白11A2抗体Defects in COL11A2 are the cause of autosomal recessive otospondylomegaepiphyseal dysplasia (OSMED) [MIM:215150]. OSMED is a skeletal dysplasia accompanied by severe hearing loss. The phenotype overlaps that of autosomal dominant skeletal disorders (Stickler and Marshall syndromes) but can be distinguished by disproportionately short limbs and lack of ocular involvement.
Defects in COL11A2 are the cause of Weissenbacher-Zweymueller syndrome (WZS) [MIM:277610]. WZS is an autosomal dominant disorder allelic with STL3 and OSMED. WZS is also referred to as heterozygous OSMED.
Defects in COL11A2 are the cause of deafness autosomal dominant type 13 (DFNA13) [MIM:601868]. DFNA13 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.
Defects in COL11A2 are the cause of deafness autosomal recessive type 53 (DFNB53) [MIM:609706].

Similarity:
Belongs to the fibrillar collagen family.
Contains 1 fibrillar collagen NC1 domain.
Contains 1 TSP N-terminal (TSPN) domain.

Database links:
UniProtKB/Swiss-Prot: P13942.5
Entrez Gene: 1302 Human
Omim: 120290 Human
SwissProt: P13942 Human
SwissProt: Q5JP94 Human
SwissProt: Q5STP6 Human
SwissProt: Q5SUI8 Human
SwissProt: Q7Z6C3 Human
Unigene: 390171 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品图片

Tissue/cell: human esophageal cancer; 4% Paraformaldehyde-fixed and paraffin-embedded;  Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;  Incubation: Anti-Collagen XI alpha 2 Polyclonal Antibody, Unconjugated(HZ-2924R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining