Thermo Fisher Scientific CLN3 Monoclonal Antibody (1G10)

Applications

Product Specifications

Product Specific Information

Sequence of this protein is as follows:
MGGCAGSRRR FSDSEGEETV PEPRLPLLDH QGAHWKNAVG FWLLGLCNNF SYVVMLSAAH DILSHKRTSG NQSHVDPGPT PIPHNSSSRF DCNSVSTAAV LLADILPTLV IKLLAPLGLH LLPYSPRVLV SGICAAGSFV LVAFSHSVGT SLCGVVFASI SSGLGEVTFL SLTAFYPRAV ISWWSSGTGG AGLLGALSYL GLTQAGLSPQ QTLLSMLGIP ALLLASYFLL LTSPEAQDPG GEEEAESAAR QPLIRTEAPE SKPGSSSSLS LRERWTVFKG LLWYIVPLVV VYFAEYFINQ GLFELLFFWN TSLSHAQQYR WYQMLYQAGV FASRSSLRCC RIRFTWALAL LQCLNLVFLL ADVWFGFLPS IYLVFLIILY EGLLGGAAYV NTFHNIALET SDEHREFAMA ATCISDTLGI SLSGLLALPL HDFLCQLS

Target Information

CLN3 is a highly glycosylated, hydrophobic 438-amino acid protein with six transmembrane domains. The CLN3 protein localizes to the lysosomal membrane and plays a role in lysosomal function. It may act as a chaperone involved in the folding and unfolding of other proteins, such as subunit C of the ATP synthase complex.
Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood caused by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C.
Batten disease is the most common form of neuronal ceroid lipofuscinoses (NCLs). Symptoms include progressive loss of vision, seizures, and psychomotor disturbances.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.