Patients with Alport syndrome have mutations in the gene for the alfa 5 chain of type IV collagen. This results in a lack of the alfa 5 (IV) chain from glomerular basement membrane (GBM) ａnd the skin basement membrane as well as a lack of the alfa 3 (IV) chain from GBM. Analysis of kidney biopsies ａnd skin biopsies for Alport syndrome can be tested for the presence or absence of the type IV collagen alpha-3, alpha-4 ａnd alpha-5 chains (COL4A3, COL4A4, COL4A5).

Alport綜合征患者為Ⅳ型膠原α5鏈的基因突變。這樣的結(jié)果在一個缺乏α5（Ⅳ）從腎小球基底膜（GBM）鏈和皮膚基底膜以及缺乏α3（Ⅳ）鏈從GBM。Alport綜合征的皮膚活檢和腎活檢分析可用于檢測Ⅳ型膠原α-3的存在或不存在下，α4和alpha-5鏈（COL4A3，COL4A4，COL4A5)

免疫組化染色試劑盒對Alport綜合征Ⅳ型膠原α5鏈的基因突變的間接檢測。結(jié)果不得用于臨床診斷和病人的管理.

試劑盒組成

200小鼠單克隆抗體µLⅣ型膠原α1鏈（單克隆抗體1）

200小鼠單克隆抗體µLⅣ型膠原α3鏈（單克隆抗體3）

100大鼠單克隆抗體µLⅣ型膠原α5鏈（單克隆抗體5）

1 x 23毫升甘氨酸/尿素溶液