Anti-SGSH/HSS 抗体 (ab96029)
Key features and details
- Rabbit polyclonal to SGSH/HSS
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
製品の概要
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製品名
Anti-SGSH/HSS antibody
SGSH/HSS 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to SGSH/HSS -
由来種
Rabbit -
アプリケーション
適用あり: WB, IHC-Pmore details -
種交差性
交差種: Human -
免疫原
Recombinant fragment corresponding to Human SGSH/HSS aa 318-466.
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ポジティブ・コントロール
- Molt-4 whole cell lysate, OVCAR3 xenograft, A431 cells
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特記事項
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
バッファー
pH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 1.21% Tris, 0.75% Glycine, 10% Glycerol (glycerin, glycerine) -
Concentration information loading...
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精製度
Immunogen affinity purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
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Related Products
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab96029の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
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WB |
1/500 - 1/3000. Predicted molecular weight: 57 kDa.
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IHC-P |
1/100 - 1/500.
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特記事項 |
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WB
1/500 - 1/3000. Predicted molecular weight: 57 kDa. |
IHC-P
1/100 - 1/500. |
ターゲット情報
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関連疾患
Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; 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. -
配列類似性
Belongs to the sulfatase family. -
翻訳後修飾
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. -
細胞内局在
Lysosome. - Information by UniProt
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参照データベース
- Entrez Gene: 6448 Human
- Omim: 605270 Human
- SwissProt: P51688 Human
- Unigene: 31074 Human
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別名
- Heparan sulfate sulfatase antibody
- Heparan sulphate sulphatase antibody
- HSS antibody
see all
画像
プロトコール
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (2)
ab96029 は 2 報の論文で使用されています。
- Duncan FJ et al. Broad functional correction of molecular impairments by systemic delivery of scAAVrh74-hSGSH gene delivery in MPS IIIA mice. Mol Ther 23:638-47 (2015). ICC/IF . PubMed: 25592334
- Boado RJ et al. Insulin receptor antibody-sulfamidase fusion protein penetrates the primate blood-brain barrier and reduces glycosoaminoglycans in Sanfilippo type A cells. Mol Pharm 11:2928-34 (2014). WB . PubMed: 24949884