The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
应用说明WB: Use at a concentration of 1-5 µg/ml.
This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.
Not yet tested in other applications. Optimal dilutions/concentrations should be determined by the end user.
功能Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.
疾病相关Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3) [MIM:204200]; also known as Batten disease. A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3.
序列相似性Belongs to the battenin family.
翻译后修饰Highly glycosylated. Farnesylation is important for trafficking to lysosomes.