ELISA/assay

Human Battenin (CLN3) ELISA Kit

MBS7206914

48-Strip-Wells

440 USD

ELISA Kit

Human Battenin (CLN3) ELISA Kit

[Battenin (CLN3)]

[battenin isoform a; Battenin; battenin; batten disease protein; ceroid-lipofuscinosis, neuronal 3; Batten disease protein; Protein CLN3]

[CLN3]

[CLN3; CLN3; BTS; JNCL; BTS]

CLN3_HUMAN

Human

This assay has high sensitivity and excellent specificity for detection of CLN3. No significant cross-reactivity or interference between CLN3 and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between CLN3 and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

Samples: Serum, plasma, cell culture supernatants, body fluid and tissue homogenate. Assay Type: Quantitative Competitive. Sensitivity: 1.0 pg/mL

Neurobiology

Intended Uses: This CLN3 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human CLN3. Principle of the Assay: CLN3 ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-CLN3 antibody and an CLN3-HRP conjugate. The assay sample and buffer are incubated together with CLN3-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the CLN3 concentration since CLN3 from samples and CLN3-HRP conjugate compete for the anti-CLN3 antibody binding site. Since the number of sites is limited, as more sites are occupied by CLN3 from the sample, fewer sites are left to bind CLN3-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The CLN3 concentration in each sample is interpolated from this standard curve.

109698601

NP_001035897.1

NM_001042432.1

37,969 Da

Lysosome Pathway (99052); Lysosome Pathway (96865)

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. [provided by RefSeq, Jul 2008]

CLN3: Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3); 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. Belongs to the battenin family. 5 isoforms of the human protein are produced by alternative splicing. Protein type: Mitochondrial; Membrane protein, integral; Chaperone; Autophagy; Vesicle; Membrane protein, multi-pass; Apoptosis. Chromosomal Location of Human Ortholog: 16p12.1. Cellular Component: Golgi apparatus; neuron projection; Golgi stack; mitochondrion; lysosomal membrane; endoplasmic reticulum; lysosome; early endosome; integral to membrane; autophagic vacuole; trans-Golgi network; caveola; lipid raft; Golgi membrane; synaptic vesicle; cytoplasm; late endosome; plasma membrane; integral to endoplasmic reticulum membrane; nucleus. Molecular Function: protein binding; unfolded protein binding. Biological Process: sphingomyelin metabolic process; autophagic vacuole fusion; macroautophagy; glucosylceramide metabolic process; negative regulation of macroautophagy; amyloid precursor protein catabolic process; neurotransmitter metabolic process; vesicle transport along microtubule; protein catabolic process; negative regulation of neuron apoptosis; neuromuscular process controlling balance; associative learning; amino acid metabolic process; negative regulation of proteolysis; regulation of action potential; receptor-mediated endocytosis; cytosolic calcium ion homeostasis; vacuolar transport; arginine transport; globoside metabolic process; galactosylceramide metabolic process; lysosome organization and biogenesis; ionotropic glutamate receptor signaling pathway; negative regulation of catalytic activity; lysosomal lumen acidification; protein processing; ceramide metabolic process; negative regulation of apoptosis. Disease: Ceroid Lipofuscinosis, Neuronal, 3

48-Strip-Wells

440 USD

96-Strip-Wells

640

5x96-Strip-Wells

2895

10x96-Strip-Wells

5415