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Human Matrilin-3 Affinity Purified Polyclonal Ab (25 UG)

货号: AF3017-SP 基本售价: 1378.1 元 规格: -

产品信息

概述
货号AF3017-SP
别名B14.5b; CI-B14.5b; complex I subunit B14.5b; Complex I-B14.5b; EDM5; HLC-1NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2 (14.5kD, B14.5b); HOA; Human lung cancer oncogene 1 protein; NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa; NADH dehydrogenase [ubiquinone] 1 subunit C2; NADHDH2; NADH-ubiquinone oxidoreductase subunit B14.5b; NDUFC2
反应种属Human
应用Western Blot(0.1 µg/mL)
目标/特异性Detects human Matrilin-3 in direct ELISAs and Western blots. In Western blots, approximately 50% cross-reactivity with recombinant mouse Matrilin-3 is observed and less than 1% cross-reactivity with recombinant human (rh) Matrilin-2 and rhMatrilin-4 is observed.
使用方法Western Blot: 0.1 µg/mL
Blockade of Receptor-ligand Interaction: In a functional ELISA, 0.03-0.1 µg/mL of this antibody will block 50% of the binding of 50 ng/mL of Recombinant Human Matrilin-3 (Catalog # 3017-MN) to immobilized Recombinant Human COMP/Thrombospondin-5 (Catalog # 3134-CP) coated at 2 µg/mL (100 µL/well). At 0.5 μg/mL, this antibody will block >90% of the binding.
来源Polyclonal Goat IgG
产品组分
性能
供应商R&D Systems
Entrez Gene IDs4718 (Human)
纯化方式Antigen Affinity-purified
免疫原Mouse myeloma cell line NS0-derived recombinant human Matrilin-3
内毒素水平<0.10 EU per 1 μg of the antibody by the LAL method.
生物活性Human
标记Unconjugated
溶解方法Reconstitute at 0.2 mg/mL in sterile PBS.
背景

Matrilin-3 is a 50‑60 kDa extracellular matrix protein that belongs to the superfamily of von Willebrand factor A (VWA) containing proteins. It is primarily expressed in cartilage and functions as a bridging component between proteins of the collagenous matrix (1‑3). The human Matrilin-3 cDNA encodes a 486 amino acid (aa) precursor with a 28 aa signal sequence, an N-terminal VWA domain, four tandem EGF-like repeats, and a C-terminal coiled‑coil domain (4). The Matrilins differ in the number of VWA domains (one or two) and EGF-like repeats (one, three, four, or ten) they contain. Human Matrilin-3 shares 82% aa sequence identity with mouse Matrilin-3. Within the first VWA domain, human Matrilin-3 shares approximately 55% aa sequence identity with human Matrilin-1, -2, and -4. The coiled‑coil domain of Matrilin-3 mediates disulfide-linked homo-oligomerization, with tetramer formation being the most dominant (5‑7). It can also assemble into hetero-oligomers with Matrilin-1 (5‑7). Matrilin-3 is more plentiful than Matrilin-1 in the proliferative zone of the growth plate, whereas the reverse is true in the maturation zone (5). Matrilin-3 interacts directly with Collagen IX and COMP (8, 9). In the absence of Collagen IX, the expression of Matrilin-3 is unchanged, although it is retained inside chondrocytes and is not incorporated into the matrix (9). Matrilin-3 also associates with smaller cartilage fibrils independent of Collagen IX (9). Matrilin-3 knockout mice do not display any obvious abnormalities, suggesting that other molecules may compensate for the lack of Matrilin-3 (10). In contrast, intracellular retention of Matrilin-3 with particular point mutations in the VWA domain results in multiple epiphyseal dysplasia (11‑13). A point mutation in the first EGF-like repeat which has been linked to hand osteoarthritis does not prevent Matrilin-3 secretion (13).

运输条件Blue Ice
存放说明4℃
参考文献
  1. Wagener, R. et al. (2005) FEBS Lett. 579:3323.
  2. Deak, F. et al. (1999) Matrix Biol. 18:55.
  3. Whittaker, C.A. and R.O. Hynes (2002) Mol. Biol. Cell 13:3369. 
  4. Belluoccio, D. et al. (1998) Genomics 53:391.
  5. Zhang, Y. and Q. Chen (2000) J. Biol. Chem. 275:32628.
  6. Klatt, A.R. et al. (2000) J. Biol. Chem. 275:3999.
  7. Frank, S. et al. (2002) J. Biol. Chem. 277:19071.
  8. Mann, H.H. et al. (2004) J. Biol. Chem. 279:25294.
  9. Budde, B. et al. (2005) Mol. Cell. Biol. 25:10465.
  10. Ko, Y. et al. (2004) Mol. Cell. Biol. 24:1691.
  11. Jackson, G.C. et al. (2004) J. Med. Genet. 41:52.
  12. Cotterill, S.L. et al. (2005) Hum. Mutat. 26:557.
  13. Otten, C. et al. (2005) J. Med. Genet. 42:774.