Product Name	CLN3
Chinese Name	神经细胞蜡样质脂褐质沉积病蛋白CLN3抗体
Alias	Batten disease protein; Battenin; BTS; Ceroid lipofuscinosis neuronal 3; Ceroid lipofuscinosis neuronal 3 juvenile (Batten Spielmeyer Vogt disease); Ceroid lipofuscinosis neuronal 3 juvenile; CLN 3; MGC102840; Protein CLN3; CLN3_HUMAN.
Research Area	Cell biology  Neurobiology
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, Cow, Horse, Rabbit, Monkey, )
Applications	WB=1:500-2000 ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	48kDa
Cellular localization	cytoplasmic The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human CLN3: 75-140/438
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. Function: Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. Subunit: Interacts with DCTN1 and KIF3A. Interacts with RAB7A and RILP. Subcellular Location: Lysosome membrane; Multi-pass membrane protein. Late endosome Post-translational modifications: Highly glycosylated. Farnesylation is important for trafficking to lysosomes. DISEASE: 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. Similarity: Belongs to the battenin family. SWISS: Q13286 Gene ID: 1201 Database links: Entrez Gene: 1201 Human Entrez Gene: 12752 Mouse Entrez Gene: 293485 Rat Omim: 607042 Human SwissProt: Q13286 Human SwissProt: Q61124 Mouse Unigene: 534667 Human Unigene: 268930 Mouse
Product Picture	Sample: Bone (Mouse) Lysate at 40 ug Primary: Anti-CLN3 (SL8016R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 48 kD Observed band size: 50 kD Sample: Heart (Mouse) Lysate at 40 ug Primary: Anti- CLN3 (SL8016R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 48 kD Observed band size: 50 kD

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