Anti-PSAP 抗体 (ab180751)
Key features and details
- Rabbit polyclonal to PSAP
- Suitable for: ICC/IF, IHC-P, WB
- Reacts with: Human
- Isotype: IgG
リコンビナント抗体で、ロット間での高い再現性を実現
- 異なるロット間での安定した再現性
- 容易なスケールアップ
- 評価試験による特異性の確認済み
- 倫理基準に準拠 - アニマル・フリーの生産
製品の概要
-
製品名
Anti-PSAP antibody
PSAP 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to PSAP -
由来種
Rabbit -
アプリケーション
適用あり: ICC/IF, IHC-P, WBmore details -
種交差性
交差種: Human -
免疫原
Recombinant fragment corresponding to Human PSAP aa 63-310.
Sequence:CDICKDVVTAAGDMLKDNATEEEILVYLEKTCDWLPKPNMSASCKEIVDS YLPVILDIIKGEMSRPGEVCSALNLCESLQKHLAELNHQKQLESNKIPEL DMTEVVAPFMANIPLLLYPQDGPRSKPQPKDNGDVCQDCIQMVTDIQTAV RTNSTFVQALVEHVKEECDRLGPGMADICKNYISQYSEIAIQMMMHMQPK EICALVGFCDEVKEMPMQTLVPAKVASKNVIPALELVEPIKKHEVPAK
Database link: P07602 -
ポジティブ・コントロール
- Extracts of HEK293 cells.
-
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
-
製品の状態
Liquid -
保存方法
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
バッファー
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 49% PBS, 50% Glycerol -
Concentration information loading...
-
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
-
Compatible Secondaries
-
Isotype control
-
Positive Controls
-
Recombinant Protein
-
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ab180751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 |
---|---|---|
ICC/IF |
Use at an assay dependent concentration.
|
|
IHC-P |
Use at an assay dependent concentration.
ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody. |
|
WB |
1/500 - 1/2000. Predicted molecular weight: 58 kDa.
|
特記事項 |
---|
ICC/IF
Use at an assay dependent concentration. |
IHC-P
Use at an assay dependent concentration. ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody. |
WB
1/500 - 1/2000. Predicted molecular weight: 58 kDa. |
ターゲット情報
-
機能
The lysosomal degradation of sphingolipids takes place by the sequential action of specific hydrolases. Some of these enzymes require specific low-molecular mass, non-enzymic proteins: the sphingolipids activator proteins (coproteins).
Saposin-A and saposin-C stimulate the hydrolysis of glucosylceramide by beta-glucosylceramidase (EC 3.2.1.45) and galactosylceramide by beta-galactosylceramidase (EC 3.2.1.46). Saposin-C apparently acts by combining with the enzyme and acidic lipid to form an activated complex, rather than by solubilizing the substrate.
Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). Saposin-B forms a solubilizing complex with the substrates of the sphingolipid hydrolases.
Saposin-D is a specific sphingomyelin phosphodiesterase activator (EC 3.1.4.12). -
関連疾患
Defects in PSAP are the cause of combined saposin deficiency (CSAPD) [MIM:611721]; also known as prosaposin deficiency. CSAPD is due to absence of all saposins, leading to a fatal storage disorder with hepatosplenomegaly and severe neurological involvement.
Defects in PSAP saposin-B region are the cause of leukodystrophy metachromatic due to saposin-B deficiency (MLD-SAPB) [MIM:249900]. MLD-SAPB is an atypical form of metachromatic leukodystrophy. It is characterized by tissue accumulation of cerebroside-3-sulfate, demyelination, periventricular white matter abnormalities, peripheral neuropathy. Additional neurological features include dysarthria, ataxic gait, psychomotr regression, seizures, cognitive decline and spastic quadriparesis.
Defects in PSAP saposin-C region are the cause of atypical Gaucher disease (AGD) [MIM:610539]. Affected individuals have marked glucosylceramide accumulation in the spleen without having a deficiency of glucosylceramide-beta glucosidase characteristic of classic Gaucher disease, a lysosomal storage disorder.
Defects in PSAP saposin-A region are the cause of atypical Krabbe disease (AKRD) [MIM:611722]. AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease.
Note=Defects in PSAP saposin-D region are found in a variant of Tay-Sachs disease (GM2-gangliosidosis). -
配列類似性
Contains 2 saposin A-type domains.
Contains 4 saposin B-type domains. -
翻訳後修飾
This precursor is proteolytically processed to 4 small peptides, which are similar to each other and are sphingolipid hydrolase activator proteins.
N-linked glycans show a high degree of microheterogeneity.
The one residue extended Saposin-B-Val is only found in 5% of the chains. -
細胞内局在
Lysosome. - Information by UniProt
-
参照データベース
- Entrez Gene: 5660 Human
- Omim: 176801 Human
- SwissProt: P07602 Human
- Unigene: 523004 Human
-
別名
- A1 activator antibody
- Cerebroside sulfate activator antibody
- Co-beta-glucosidase antibody
see all
画像
プロトコール
データシートおよび資料
-
SDS download
-
Datasheet download
参考文献 (2)
ab180751 は 2 報の論文で使用されています。
- van Leent MMT et al. Prosaposin mediates inflammation in atherosclerosis. Sci Transl Med 13:N/A (2021). PubMed: 33692130
- Patten DA et al. Mitochondrial adaptation in human mesenchymal stem cells following ionizing radiation. FASEB J 33:9263-9278 (2019). PubMed: 31112400