克隆類(lèi)型 : |
Rabbit Polyclonal IgG |
免 疫 原 : |
Peptide derived from Hu EVC2. |
純化方式 : |
Immunogen affinity purified |
緩 沖 液 : |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
產(chǎn)品描述 : |
EVC2 is an integral membrane protein that plays a vital role in bone formation and skeletal development. Defects in EVC2 are a cause of Ellis-van Creveld syndrome (EVC), also known as chondroectodermal dysplasia. EVC is an autosomal recessive disorder characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia and cardiac anomalies. Patients manifest short-limb dwarfism, short ribs, postaxial polydactyly and dysplastic nails and teeth. |
適用物種 : |
Hu |
應(yīng)用說(shuō)明 : |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
別 名 : |
Ellis van Creveld syndrome 2; LBN; Limbin; LBN_Hu. |
保存條件 : |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
標(biāo) 記 物 : |
PLLABS公司可供應(yīng)生物素、辣根過(guò)氧化物酶、熒光素標(biāo)記抗體 |
產(chǎn)品說(shuō)明 : |
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