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Anti-Collagen II alpha 1/ChondrocalcinⅡ型胶原α1蛋白/软骨钙素抗体
点击次数:366发布时间:2012/12/26 10:15:59
更新日期:2024/9/5 14:43:40
所 在 地:其它
产品型号:BY-4850R
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货号:BY-4850R
英文名称:Anti-Collagen II alpha 1/Chondrocalcin
中文名称:Ⅱ型胶原α1蛋白/软骨钙素抗体
其他名称:Collagen II alpha 1; COL2A1; COL2A1 protein; collagen, type II, alpha 1; collagen alpha-1(II); type II collagen; alpha-1 type II collagen; alpha1 type II collagen; Col2a1; AOM; Cartilage collagen; Chondrocalcin; COL11A3; Collagen alpha 1(II) chain precursor; Collagen II alpha 1 polypeptide; Collagen type II alpha 1 (primary osteoarthritis spondyloepiphyseal dysplasia congenital); MGC131516; SEDC.
抗体来源:Rabbit
克隆类型:polyclonal
蛋白分子量:predicted molecular weight: 27kDa
纯化方法:affinity purified by Protein A
交叉反应:hu, mo, rat, Rb, cow, dog, chk, Gpig, pig
产品介绍:Type II collagen is specific for cartilaginous tissues. It is essential for the normal embryonic development of the skeleton, for linear growth and for the ability of cartilage to resist compressive forces.The extensive family of COL gene products (collagens) is composed of several chain types, including fibril-forming interstitial collagens (types I, II, III and V) and basement membrane collagens (type IV), each type containing multiple isoforms. Collagens are fibrous, extracellular matrix proteins with high tensile strength and are the major components of connective tissue, such as tendons and cartilage. All collagens contain a triple helix domain and frequently show lateral self-association in order to form complex connective tissues. Several collagens also play a role in cell adhesion, important for maintaining normal tissue architecture and function. In cartilage, Collagen Type II constitutes the bulk of the fibril. Sensitization with Collagen Type II induces an erosive polyarthritis in rats, mice and higher primates which can resemble rheumatoid arthritis and relapsing polychrondritis.Function : Type II collagen is specific for cartilaginous tissues. It is essential for the normal embryonic development of the skeleton, for linear growth and for the ability of cartilage to resist compressive forces.Subunit : Homotrimers of alpha 1(II) chains.Subcellular Location : Secreted, extracellular space, extracellular matrix.Tissue Specificity : Isoform 2 is highly expressed in juvenile chondrocyte and low in fetal chondrocyte.Post-translational modifications : Proline residues at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. Proline residues at the second position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some of the chains.The N-telopeptide is covalently linked to the helical COL2 region of alpha 1(IX), alpha 2(IX) and alpha 3(IX) chain. The C-telopeptide is covalently linked to an another site in the helical region of alpha 3(IX) COL2.DISEASE : Defects in COL2A1 are the cause of spondyloepiphyseal dysplasia congenital type (SEDC) [MIM:183900]. This disorder is characterized by disproportionate short stature and pleiotropic involvement of the skeletal and ocular systems.[DISEASE] Defects in COL2A1 are the cause of spondyloepimetaphyseal dysplasia Strudwick type (SEMD-STR) [MIM:184250]. A bone disease characterized by disproportionate short stature from birth, with a very short trunk and shortened limbs, and skeletal abnormalities including lordosis, scoliosis, flattened vertebrae, pectus carinatum, coxa vara, clubfoot, and abnormal epiphyses or metaphyses. A distinctive radiographic feature is irregular sclerotic changes, described as dappled in the metaphyses of the long bones.[DISEASE] Defects in COL2A1 are the cause of achondrogenesis type 2 (ACG2) [MIM:200610]; also known as achondrogenesis-hypochondrogenesis type II. ACG2 is a disease characterized by the absence of ossification in the vertebral column, sacrum and pubic bones.[DISEASE] Defects in COL2A1 are the cause of Legg-Calve-Perthes disease (LCPD) [MIM:150600]; also known as Legg-Perthes disease or Perthes disease. LCPD is characterized by loss of circulation to the femoral head, resulting in avascular necrosis in a growing child. Clinical pictures of the disease vary, depending on the phase of disease progression through ischemia, revascularization, fracture and collapse, and repair and remodeling of the bone. [DISEASE] Defects in COL2A1 are the cause of Kniest dysplasia (KD) [MIM:156550]; also known as Kniest syndrome or metatropic dwarfism type II. KD is a moderately severe chondrodysplasia phenotype that results from mutations in the COL2A1 gene. Characteristics of the disorder include a short trunk and extremities, mid-face hypoplasia, cleft palate, myopia, retinal detachment, and hearing loss.Similarity : Belongs to the fibrillar collagen family.Contains 1 fibrillar collagen NC1 domain.Contains 1 VWFC domain.Ⅱ型胶原是软骨基质中的一种结构蛋白;Ⅱ型胶原不与Ⅰ, Ⅲ, Ⅳ, Ⅴ, Ⅵ胶原以及其它血清蛋白或非胶原性细胞外相关蛋白起交叉反应。II型胶原*先发现于软骨基质中,在眼睛中也有少量存在。组成II型胶原的纤维较I型胶原更纤细。该抗体可以特异性识别II型胶原,与其他类型的胶原无交叉反应,抗II型胶原抗体-主要用于Ⅱ型胶原分布及变态反应方面及良/恶性肿瘤的细胞外基质方面的研究。