Anti-CLN5 Antibody (CAB12886)
- SKU:
- CAB12886
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-CLN5 Antibody |
| 抗体コード: | CAB12886 |
| 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 96-407 of human CLN5 (NP_006484.1). |
| 申し込み: | WB IHC |
| 推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | Mouse heart, Rat heart |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 96-407 of human CLN5 (NP_006484.1). |
| 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: | IPSR RHWP VPYK RFDF RPKP DPYC QAKY TFCP TGSP IPVM EGDD DIEV FRLQ APVW EFKY GDLL GHLK IMHD AIGF RSTL TGKN YTME WYEL FQLG NCTF PHLR PEMD APFW CNQG AACF FEGI DDVH WKEN GTLV QVAT ISGN MFNQ MAKW VKQD NETG IYYE TWNV KASP EKGA ETWF DSYD CSKF VLRT FNKL AEFG AEFK NIET NYTR IFLY SGEP TYLG NETS VFGP TGNK TLGL AIKR FYYP FKPH LPTK EFLL SLLQ IFDA VIVH KQFY LFYN FEYW FLPM KFPF IKIT YEEI PLPI RNKT LSGL |
| 遺伝子ID: | 1203 |
| Uniprot: | O75503 |
| セルラーロケーション: | Lysosome |
| 計算された分子量: | 41kDa |
| 観察された分子量: | 41kDa |
| 同義語: | CLN5, NCL |
| バックグラウンド: | 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. |
| UniProt Protein Function: | CLN5: Defects in CLN5 are the cause of neuronal ceroid lipofuscinosis type 5 (CLN5); also known as Finnish variant late-infantile neuronal ceroid lipofuscinosis (vLINCL). 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 5 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Belongs to the CLN5 family. |
| UniProt Protein Details: | Protein type:Membrane protein, integral Chromosomal Location of Human Ortholog: 13q22.3 Cellular Component: endoplasmic reticulum; Golgi apparatus; integral to membrane; lysosomal membrane; lysosome; perinuclear region of cytoplasm Molecular Function:mannose binding; protein binding Biological Process: brain development; lysosomal lumen acidification; neurogenesis; retrograde transport, endosome to Golgi; signal peptide processing Disease: Ceroid Lipofuscinosis, Neuronal, 5 |
| 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: | O75503 |
| NCBI GenInfo Identifier: | 187608866 |
| NCBI Gene ID: | 1203 |
| NCBI Accession: | O75503.2 |
| UniProt Secondary Accession: | O75503,B3KQK7, |
| UniProt Related Accession: | O75503 |
| Molecular Weight: | 41kDa |
| NCBI Full Name: | Ceroid-lipofuscinosis neuronal protein 5 |
| NCBI Synonym Full Names: | ceroid-lipofuscinosis, neuronal 5 |
| NCBI Official Symbol: | CLN5 |
| NCBI Official Synonym Symbols: | NCL |
| NCBI Protein Information: | ceroid-lipofuscinosis neuronal protein 5 |
| UniProt Protein Name: | Ceroid-lipofuscinosis neuronal protein 5 |
| Protein Family: | Cln5-like protein |
| UniProt Gene Name: | CLN5 |
 | Western blot analysis of extracts of various cell lines, using CLN5 antibody (CAB12886) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
 | Immunohistochemistry of paraffin-embedded human appendix using CLN5 antibody (CAB12886) at dilution of 1:150 (40x lens). |
View AllClose