製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号P07954
    • 生物種Human
    • 配列MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAMTRIK AAMPRIYELA AGGTAVGTGL NTRIGFAEKV AAKVAALTGL PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR FLGSGPRSGL GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL MLVTALNPHI GYDKAAKIAK TAHKNGSTLK ETAIELGYLT AEQFDEWVKP KDMLGPK
    • 分子量50 kDa
    • 領域44 to 510

特性

Our Abpromise guarantee covers the use of ab116496 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • アプリケーション

    SDS-PAGE

  • 精製度> 95 % SDS-PAGE.
    ab116496 was purified by proprietary chromatographic techniques and filter sterilized.
  • 製品の状態Liquid
  • 備考Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped at 4°C. Please see notes section.

    pH: 8.00
    Constituent: 0.32% Tris HCl

関連情報

  • 別名
    • FH
    • Fumarase
    • Fumarate hydratase
    • Fumarate hydratase mitochondrial
    • Fumarate hydratase, mitochondrial
    • FUMH_HUMAN
    • HLRCC
    • LRCC
    • MCL
    • MCUL 1
    • MCUL1
    • MS709
    • Multiple hereditary cutaneous leiomyomata
    see all
  • 機能Also acts as a tumor suppressor.
  • パスウェイCarbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
  • 関連疾患Defects in FH are the cause of fumarase deficiency (FHD) [MIM:606812]; also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.
    Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).
    Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].
  • 配列類似性Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
  • 細胞内局在Cytoplasm and Mitochondrion.
  • Information by UniProt

Recombinant Human FH protein (ab116496) 使用論文

ab116496 has not yet been referenced specifically in any publications.

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