Anti-SGSH Antibody (CAB8148)
- SKU:
- CAB8148
- Product type:
- Antibody
- Application:
- WB
- Application:
- IHC
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | SGSH Rabbit Polyclonal Antibody |
| 抗体コード: | CAB8148 |
| 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 273-502 of human SGSH (NP_000190.1). |
| 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: | MCF7, 22Rv1, HepG2, Mouse liver, Mouse testis |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1). |
| 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: | DNGI PFPS GRTN LYWP GTAE PLLV SSPE HPKR WGQV SEAY VSLL DLTP TILD WFSI PYPS YAIF GSKT IHLT GRSL LPAL EAEP LWAT VFGS QSHH EVTM SYPM RSVQ HRHF RLVH NLNF KMPF PIDQ DFYV SPTF QDLL NRTT AGQP TGWY KDLR HYYY RARW ELYD RSRD PHET QNLA TDPR FAQL LEML RDQL AKWQ WETH DPWV CAPD GVLE EKLS PQCQ PLHN EL |
| 遺伝子ID: | 6448 |
| Uniprot: | P51688 |
| セルラーロケーション: | Lysosome |
| 計算された分子量: | 56kDa |
| 観察された分子量: | 63kDa |
| 同義語: | SGSH, HSS, MPS3A, SFMD |
| バックグラウンド: | This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. |
| UniProt Protein Function: | SGSH: Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A); also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. Belongs to the sulfatase family. |
| UniProt Protein Details: | Protein type:Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.10.1.1 Chromosomal Location of Human Ortholog: 17q25.3 Cellular Component: lysosomal lumen Molecular Function:N-sulfoglucosamine sulfohydrolase activity; metal ion binding; sulfuric ester hydrolase activity; catalytic activity Biological Process: proteoglycan metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis Disease: Mucopolysaccharidosis, Type Iiia |
| NCBI Summary: | This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P51688 |
| NCBI GenInfo Identifier: | 4506919 |
| NCBI Gene ID: | 6448 |
| NCBI Accession: | NP_000190.1 |
| UniProt Secondary Accession: | P51688,A8K5E2, |
| UniProt Related Accession: | P51688 |
| Molecular Weight: | 56,695 Da |
| NCBI Full Name: | N-sulphoglucosamine sulphohydrolase |
| NCBI Synonym Full Names: | N-sulfoglucosamine sulfohydrolase |
| NCBI Official Symbol: | SGSH |
| NCBI Official Synonym Symbols: | HSS; SFMD; MPS3A |
| NCBI Protein Information: | N-sulphoglucosamine sulphohydrolase; sulphamidase; heparan sulfate sulfatase; sulfoglucosamine sulfamidase; mucopolysaccharidosis type IIIA |
| UniProt Protein Name: | N-sulphoglucosamine sulphohydrolase |
| UniProt Synonym Protein Names: | Sulfoglucosamine sulfamidase; Sulphamidase |
| Protein Family: | N-sulphoglucosamine sulphohydrolase |
| UniProt Gene Name: | SGSH |
| UniProt Entry Name: | SPHM_HUMAN |
 | Western blot analysis of extracts of various cell lines, using SGSH antibody (CAB8148) 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: 10s. |
 | Immunohistochemistry of paraffin-embedded human liver using SGSH antibody (CAB8148) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human stomach using SGSH antibody (CAB8148) at dilution of 1:100 (40x lens). |
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