Anti-CLN3 Antibody (CAB1931)
- SKU:
- CAB1931
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Signal Transduction
Description
抗体名: | Anti-CLN3 Antibody |
抗体コード: | CAB1931 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human CLN3 (NP_001035897.1). |
申し込み: | WB IHC |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:100 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | Mouse liver, Rat liver |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human CLN3 (NP_001035897.1). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | MGGC AGSR RRFS DSEG EETV PEPR LPLL DHQG AHWK NAVG FWLL GLCN NFSY VVML SAAH DILS HKRT SGNQ SHVD PGPT PIPH NSSS RFDC NSVS TAAV LLAD ILPT LVIK LLAP LGLH LLPY SPRV LVSG ICAA GSFV LVAF SHSV GTSL CGVV FASI SSGL GEVT FLSL TAFY PRAV ISWW SSGT GGAG LLGA LSYL GLTQ AGLS PQQT LLSM LGIP ALLL ASYF LLLT SPEA QDPG GEEE AESA ARQP LIRT EAPE SKPG SSSS LSLR ERWT VFKG |
遺伝子ID: | 1201 |
Uniprot: | Q13286 |
セルラーロケーション: | Late endosome, Lysosome membrane, Multi-pass membrane protein |
計算された分子量: | 28kDa/34kDa/37kDa/42kDa/45kDa/47kDa |
観察された分子量: | 48kDa |
同義語: | CLN3, BTN1, BTS, JNCL, battenin |
バックグラウンド: | 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. |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Mitochondrial; Vesicle; Autophagy; Chaperone; Apoptosis; Membrane protein, multi-pass Chromosomal Location of Human Ortholog: 16p12.1 Cellular Component: Golgi apparatus; neuron projection; Golgi stack; mitochondrion; lysosomal membrane; lysosome; endoplasmic reticulum; early endosome; integral to membrane; autophagic vacuole; trans-Golgi network; caveola; lipid raft; Golgi membrane; synaptic vesicle; late endosome; cytoplasm; 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; receptor-mediated endocytosis; regulation of action potential; 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 |
NCBI Summary: | 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] |
UniProt Code: | Q13286 |
NCBI GenInfo Identifier: | 2498243 |
NCBI Gene ID: | 1201 |
NCBI Accession: | Q13286.1 |
UniProt Related Accession: | Q13286 |
Molecular Weight: | |
NCBI Full Name: | Battenin |
NCBI Synonym Full Names: | CLN3 lysosomal/endosomal transmembrane protein, battenin |
NCBI Official Symbol: | CLN3 |
NCBI Official Synonym Symbols: | BTS; BTN1; JNCL |
NCBI Protein Information: | battenin |
UniProt Protein Name: | Battenin |
UniProt Synonym Protein Names: | Batten disease protein; Protein CLN3 |
Protein Family: | Zinc finger protein |
UniProt Gene Name: | CLN3 |
UniProt Entry Name: | CLN3_HUMAN |