Anti-CLN6 Antibody (CAB18467)
- SKU:
- CAB18467
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
Frequently bought together:
Description
| 抗体名: | Anti-CLN6 Antibody |
| 抗体コード: | CAB18467 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant protein of human CLN6. |
| 申し込み: | WB |
| 推奨希釈: | WB 1:500 - 1:2000 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: |
| 免疫原: | Recombinant protein of human CLN6. |
| 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: | Email for sequence |
| 遺伝子ID: | 54982 |
| Uniprot: | Q9NWW5 |
| セルラーロケーション: | |
| 計算された分子量: | |
| 観察された分子量: | Refer to figures |
| 同義語: | CLN4A, HsT18960, nclf, CLN6 |
| バックグラウンド: |
| UniProt Protein Function: | CLN6: Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 6 (CLN6). 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 lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 6 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 4A (CLN4A). An adult-onset 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. CLN4A has no visual involvement and is characterized by progressive myoclonic epilepsy. |
| UniProt Protein Details: | Protein type:Membrane protein, multi-pass; Membrane protein, integral Chromosomal Location of Human Ortholog: 15q23 Cellular Component: endoplasmic reticulum; endoplasmic reticulum lumen; endoplasmic reticulum membrane; integral to membrane; membrane Molecular Function:protein binding; protein homodimerization activity Biological Process: cellular macromolecule catabolic process; cholesterol metabolic process; ganglioside metabolic process; glycosaminoglycan metabolic process; locomotion during locomotory behavior; lysosomal lumen acidification; positive regulation of proteolysis; protein catabolic process; visual perception Disease: Ceroid Lipofuscinosis, Neuronal, 4a, Autosomal Recessive; Ceroid Lipofuscinosis, Neuronal, 6 |
| NCBI Summary: | This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function. [provided by RefSeq, Oct 2008] |
| UniProt Code: | Q9NWW5 |
| NCBI GenInfo Identifier: | 8923532 |
| NCBI Gene ID: | 54982 |
| NCBI Accession: | NP_060352.1 |
| UniProt Secondary Accession: | Q9NWW5,Q6IAB1, Q96SR0, A8K560, B4DDH6, |
| UniProt Related Accession: | Q9NWW5 |
| Molecular Weight: | 39,484 Da |
| NCBI Full Name: | ceroid-lipofuscinosis neuronal protein 6 |
| NCBI Synonym Full Names: | ceroid-lipofuscinosis, neuronal 6, late infantile, variant |
| NCBI Official Symbol: | CLN6 |
| NCBI Official Synonym Symbols: | nclf; CLN4A; HsT18960 |
| NCBI Protein Information: | ceroid-lipofuscinosis neuronal protein 6 |
| UniProt Protein Name: | Ceroid-lipofuscinosis neuronal protein 6 |
| Protein Family: | Ceroid-lipofuscinosis neuronal protein |
| UniProt Gene Name: | CLN6 |
| UniProt Entry Name: | CLN6_HUMAN |