Recombinant Human PEX19 protein (ab111623)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE, MS
製品の詳細
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製品名
Recombinant Human PEX19 protein -
精製度
> 90 % SDS-PAGE.
ab111623 is purified using conventional chromatography techniques. -
発現系
Escherichia coli -
アクセッション番号
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タンパク質長
Full length protein -
Animal free
No -
由来
Recombinant -
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生物種
Human -
配列
MGSSHHHHHHSSGLVPRGSHMAAAEEGCSVGAEADRELEELLESALDDFD KAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELA SQATAEFEKAMKELAEEEPHLVEQFQKLSEAAGRVGSDMTSQQEFTSCLK ETLSGLAKNATDLQNSSMSEEELTKAMEGLGMDEGDGEGNILPIMQSIMQ NLLSKDVLYPSLKEITEKYPEWLQSHRESLPPEQFEKYQEQHSVMCKICE QFEAETPTDSETTQKARFEMVLDLMQQLQDLGHPPKELAGEMPPGLNFDL DALNLSGPPGASGEQC -
予測される分子量
35 kDa including tags -
領域
1 to 296 -
タグ
His tag N-Terminus
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関連製品
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Related Products
特性
Our Abpromise guarantee covers the use of ab111623 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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アプリケーション
SDS-PAGE
Mass Spectrometry
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質量分析
MALDI-TOF -
製品の状態
Liquid -
Concentration information loading...
前処理および保存
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保存方法および安定性
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
pH: 8.00
Constituents: 0.32% Tris HCl, 10% Glycerol (glycerin, glycerine)
関連情報
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別名
- 33 kDa housekeeping protein
- D1S2223E
- HK33
see all -
機能
Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53. -
組織特異性
Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form. -
関連疾患
Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:600279]; also known as PBD-CGJ. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies.
Defects in PEX19 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life. -
配列類似性
Belongs to the peroxin-19 family. -
細胞内局在
Cytoplasm. Peroxisome membrane. Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes. - Information by UniProt
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プロトコール
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
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 (0)
ab111623 は論文での使用が確認できていません。