Anti-NAGA Antibody (CAB9942)
- SKU:
- CAB9942
- Product type:
- Antibody
- Application:
- WB
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-NAGA Antibody |
| 抗体コード: | CAB9942 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 312-411 of human NAGA (NP_000253.1). |
| 申し込み: | WB IF |
| 推奨希釈: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | HL-60, Mouse liver, Mouse kidney, Mouse lung, Rat testis |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 312-411 of human NAGA (NP_000253.1). |
| 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: | IQGR RIHK EKSL IEVY MRPL SNKA SALV FFSC RTDM PYRY HSSL GQLN FTGS VIYE AQDV YSGD IISG LRDE TNFT VIIN PSGV VMWY LYPI KNLE MSQQ |
| 遺伝子ID: | 4668 |
| Uniprot: | P17050 |
| セルラーロケーション: | Lysosome |
| 計算された分子量: | 46kDa |
| 観察された分子量: | 46kDa |
| 同義語: | NAGA, D22S674, GALB |
| バックグラウンド: | NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. Mutations in NAGA have been identified as the cause of Schindler disease types I and II (type II also known as Kanzaki disease). |
| UniProt Protein Function: | NAGA: Removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. Required for the breakdown of glycolipids. Defects in NAGA are the cause of Schindler disease (SCHIND). Schindler disease is a form of NAGA deficiency characterized by early onset neuroaxonal dystrophy and neurological signs (convulsion during fever, epilepsy, psychomotor retardation and hypotonia). NAGA deficiency is typically classified in three main phenotypes: NAGA deficiency type I (Schindler disease or Schindler disease type I) with severe manifestations; NAGA deficiency type II (Kanzazi disease or Schindler disease type II) which is mild; NAGA deficiency type III (Schindler disease type III) characterized by mild-to-moderate neurologic manifestations. NAGA deficiency results in the increased urinary excretion of glycopeptides and oligosaccharides containing alpha-N-acetylgalactosaminyl moieties. Inheritance is autosomal recessive. Defects in NAGA are the cause of Kanzaki disease (KANZD); also known as NAGA deficiency type II or Schindler disease type II. Kanzaki disease is an autosomal recessive disorder characterized by late onset, angiokeratoma corporis diffusum and mild intellectual impairment. Belongs to the glycosyl hydrolase 27 family. |
| UniProt Protein Details: | Protein type:Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; EC 3.2.1.49 Chromosomal Location of Human Ortholog: 22q11 Cellular Component: lysosome; cytoplasm Molecular Function:protein homodimerization activity; alpha-galactosidase activity; alpha-N-acetylgalactosaminidase activity Biological Process: glycolipid catabolic process; carbohydrate catabolic process; glycoside catabolic process; glycosylceramide catabolic process; oligosaccharide metabolic process Disease: Schindler Disease, Type I; Kanzaki Disease |
| NCBI Summary: | NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. Mutations in NAGA have been identified as the cause of Schindler disease types I and II (type II also known as Kanzaki disease). [provided by RefSeq, Jul 2008] |
| UniProt Code: | P17050 |
| NCBI GenInfo Identifier: | 4557781 |
| NCBI Gene ID: | 4668 |
| NCBI Accession: | NP_000253.1 |
| UniProt Related Accession: | P17050 |
| Molecular Weight: | 46,565 Da |
| NCBI Full Name: | alpha-N-acetylgalactosaminidase |
| NCBI Synonym Full Names: | N-acetylgalactosaminidase, alpha- |
| NCBI Official Symbol: | NAGA |
| NCBI Official Synonym Symbols: | GALB; D22S674 |
| NCBI Protein Information: | alpha-N-acetylgalactosaminidase; alpha-galactosidase B; Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B) |
| UniProt Protein Name: | Alpha-N-acetylgalactosaminidase |
| UniProt Synonym Protein Names: | Alpha-galactosidase B |
| Protein Family: | Alpha-N-acetylgalactosaminidase |
| UniProt Gene Name: | NAGA |
| UniProt Entry Name: | NAGAB_HUMAN |
 | Western blot analysis of extracts of various cell lines, using NAGA antibody (CAB9942) at 1:1000 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: 30s. |
 | Immunofluorescence analysis of L929 cells using NAGA Rabbit pAb (CAB9942) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining. |
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