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综合征患者为Ⅳ型胶原α5链的基因突变。这样的结果在一个缺乏α5（Ⅳ）从肾小球基底膜（GBM）链和皮肤基底膜以及缺乏α3（Ⅳ）链从GBM。Alport综合征的皮肤活检和肾活检分析可用于检测Ⅳ型胶原α-3的存在或不存在下，α4和alpha-5链（COL4A3，COL4A4，COL4A5)

免疫组化染色试剂盒对Alport综合征Ⅳ型胶原α5链的基因突变的间接检测。结果不得用于临床诊断和病人的管理.

试剂盒组成

200小鼠单克隆抗体µLⅣ型胶原α1链（单克隆抗体1）

200小鼠单克隆抗体µLⅣ型胶原α3链（单克隆抗体3）

100大鼠单克隆抗体µLⅣ型胶原α5链（单克隆抗体5）

1 x 23毫升甘氨酸/尿素溶液