Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MGEFKVHRVRFFNYVPSGIRCVAYNNQSNRLAVSRTDGTVEIYNLSANYF QEKFFPGHESRATEALCWAEGQRLFSAGLNGEIMEYDLQALNIKYAMDAF GGPIWSMAASPSGSQLLVGCEDGSVKLFQITPDKIQFERNFDRQKSRILS LSWHPSGTHIAAGSIDYISVFDVKSGSAVHKMIVDRQYMGVSKRKCIVWG VAFLSDGTIISVDSAGKVQFWDSATGTLVKSHLIANADVQSIAVADQEDS FVVGTAEGTVFHFQLVPVTSNSSEKQWVRTKPFQHHTHDVRTVAHSPTAL ISGGTDTHLVFRPLMEKVEVKNYDAALRKITFPHRCLISCSKKRQLLLFQ FAHHLELWRLGSTVATGKNGDTLPLSKNADHLLHLKTKGPENIICSCISP CGSWIAYSTVSRFFLYRLNYEHDNISLKRVSKMPAFLRSALQILFSEDST KLFVASNQGALHIVQLSGGSFKHLHAFQPQSGTVEAMCLLAVSPDGNWLA ASGTSAGVHVYNVKQLKLHCTVPAYNFPVTAMAIAPNTNNLVIAHSDQQV FEYSIPDKQYTDWSRTVQKQGFHHLWLQRDTPITHISFHPKRPMHILLHD AYMFCIIDKSLPLPNDKTLLYNPFPPTNESDVIRRRTAHAFKISKIYKPL LFMDLLDERTLVAVERPLDDIIAQLPPPIKKKKFGT
    • Amino acids
      1 to 686
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab164691 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • CIRH1A
    • Cirhin
    • Cirrhosis, autosomal recessive 1A (cirhin)
    • FLJ17146
    • KIAA1988
    • NAIC
    • testis expressed gene 292
    • TEX292
    • U3 small nucleolar RNA associated protein 4 homolog
    • UTP4
    • UTP4 small subunit (SSU) processome component
    • UTP4 small subunit (SSU) processome component homolog
    • UTP4, small subunit processome component
    see all
  • Relevance
    Defects in Cirhin are the cause of North American Indian childhood cirrhosis. NAIC is a severe autosomal recessive intrahepatic cholestasis, originally described in Ojibway-Cree children from northwestern Quebec. NAIC typically presents with transient neonatal jaundice, in a child who is otherwise healthy, and progresses to biliary cirrhosis and portal hypertension. Biochemical and histopathological features suggest involvement of the bile ducts rather than of the bile canaliculi. They include elevated gamma glutamyltransferase and alkaline phosphatase levels, and, typically, marked fibrosis around bile ducts. Clinically, NAIC is distinct from other nonsyndromic familial cholestases because of its marked cholangiopathic features and severe degree of fibrosis on liver histology.
  • Cellular localization
    Nucleus; nucleolus

Images

  • ab164691 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab164691 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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