Description

  • Product name

    Recombinant Human IKK gamma/NEMO protein
    See all IKK gamma/NEMO proteins and peptides
  • Purity

    >= 85 % SDS-PAGE.

  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MNRHLWKSQLCEMVQPSGGPAADQDVLGEESPLGKPAMLHLPSEQGAPET LQRCLEENQELRDAIRQSNQILRERCEELLHFQASQREEKEFLMCKFQEA RKLVERLGLEKLDLKRQKEQALREVEHLKRCQQQMAEDKASVKAQVTSLL GELQESQSRLEAATKECQALEGRARAASEQ ARQLESEREALQQQHSVQ VDQLRMQGQSVEAALRMERQAASEEKRKLAQLQVAYHQLFQEYDNHIKSS VVGSERKRGMQLEDLKQQLQQAEEALVAKQEVIDKLKEEAEQHKIVMETV PVLKAQADIYKADFQAERQAREKLAEKKELLQEQLEQLQREYSKLKASCQ ESARIEDMRKRHVEVSQAPLPPAPAYLSSPLALPSQRRSPPEEPPDFCCP KCQYQAPDMDTLQIHVMECIE
    • Predicted molecular weight

      48 kDa
    • Amino acids

      1 to 419
    • Tags

      GST tag N-Terminus
    • Additional sequence information

      NM_001099857

Specifications

Our Abpromise guarantee covers the use of ab206008 in the following tested applications.

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

  • Applications

    SDS-PAGE

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on Dry Ice. Store at -80°C. Avoid freeze / thaw cycle.

    Constituents: 0.32% Tris, 0.87% Sodium chloride, 0.15% Beta mercaptoethanol, 10% Glycerol

General Info

  • Alternative names

    • IkB kinase associated protein 1
    • IkB kinase subunit gamma
    • Inhibitor of nuclear factor kappa B kinase subunit gamma
    • AMCBX1
    • FIP 3
    • FIP-3
    • FIP3
    • Fip3p
    • I kappa B kinase gamma
    • I-kappa-B kinase subunit gamma
    • IkB kinase gamma subunit
    • IkB kinase subunit gamma
    • IkB kinase-associated protein 1
    • Ikbkg
    • IKK gamma
    • IKK-gamma
    • IKKAP1
    • IKKG
    • IMD33
    • Incontinentia pigmenti
    • Inhibitor of kappa light polypeptide gene enhancer in B cells, kinase gamma
    • Inhibitor of kappa light polypeptide gene enhancer in B cells, kinase of, gamma
    • Inhibitor of nuclear factor kappa-B kinase subunit gamma
    • IP
    • IP1
    • IP2
    • IPD2
    • NEMO
    • NEMO_HUMAN
    • NF kappa B essential modifier
    • NF kappa B essential modulator
    • NF-kappa-B essential modifier
    • NF-kappa-B essential modulator
    • ZC2HC9
    see all
  • Function

    Regulatory subunit of the IKK core complex which phosphorylates inhibitors of NF-kappa-B thus leading to the dissociation of the inhibitor/NF-kappa-B complex and ultimately the degradation of the inhibitor. Also considered to be a mediator for TAX activation of NF-kappa-B. Could be implicated in NF-kappa-B-mediated protection from cytokine toxicity (By similarity). Essential for viral activation of IRF3.
  • Tissue specificity

    Heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • Involvement in disease

    Defects in IKBKG are the cause of ectodermal dysplasia anhidrotic with immunodeficiency X-linked (EDAID) [MIM:300291]; also known as hypohidrotic ectodermal dysplasia with immunodeficiency (HED-ID). Is a form of ectoderma dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. Characterized by absence of sweat glands, sparse scalp hair, rare conical teeth and immunological abnormalities resulting in severe infectious diseases.
    Defects in IKBKG are the cause of ectodermal dysplasia anhidrotic with immunodeficiency-osteopetrosis-lymphedema (OLEDAID) [MIM:300301].
    Defects in IKBKG are a cause of immunodeficiency NEMO-related without anhidrotic ectodermal dysplasia (NEMOID) [MIM:300584]; also called immunodeficiency without anhidrotic ectodermal dysplasia, isolated immunodeficiency or pure immunodeficiency. Patients manifest immunodeficiency not associated with other abnormalities, and resulting in increased infection susceptibility. Patients suffer from multiple episodes of infectious diseases.
    Defects in IKBKG are the cause of susceptibility to X-linked familial atypical micobacteriosis type 1 (AMCBX1) [MIM:300636]; also known as X-linked disseminated atypical mycobacterial infection type 1 or X-linked susceptibility to mycobacterial disease type 1. AMCBX1 is the X-linked recessive form of mendelian susceptibility to mycobacterial disease (MSMD). MSMD is a congenital syndrome resulting in predisposition to clinical disease caused by weakly virulent mycobacterial species, such as bacillus Calmette-Guerin vaccines and non-tuberculous, environmental mycobacteria. Patients are also susceptible to the more virulent species Mycobacterium tuberculosis.
    Defects in IKBKG are the cause of recurrent isolated invasive pneumococcal disease type 2 (IPD2) [MIM:300640]. Recurrent invasive pneumococcal disease (IPD) is defined as two episodes of IPD occurring at least 1 month apart, whether caused by the same or different serotypes or strains. Recurrent IPD occurs in at least 2% of patients in most series, making IPD the most important known risk factor for subsequent IPD.
    Defects in IKBKG are the cause of incontinentia pigmenti (IP) [MIM:308300]; formerly designed familial incontinentia pigmenti type II (IP2). IP is a genodermatosis usually prenatally lethal in males. In affected females, it causes abnormalities of the skin, hair, eyes, nails, teeth, skeleton, heart, and central nervous system. The prominent skin signs occur in four classic cutaneous stages: perinatal inflammatory vesicles, verrucous patches, a distinctive pattern of hyperpigmentation and dermal scarring.
  • Sequence similarities

    Contains 1 C2HC-type zinc finger.
  • Domain

    The leucine-zipper domain and the C2HC-type zinc-finger are essential for polyubiquitin binding and for the activation of IRF3.
  • Post-translational
    modifications

    Phosphorylation at Ser-68 attenuates aminoterminal homodimerization.
    Polyubiquitinated on Lys-285 through 'Lys-63'; the ubiquitination is mediated by NOD2 and RIPK2 and probably plays a role in signaling by facilitating interactions with ubiquitin domain-containing proteins and activates the NF-kappa-B pathway. Polyubiquitinated on Lys-399 through 'Lys-63'; the ubiquitination is mediated by BCL10, MALT1 and TRAF6 and probably plays a role in signaling by facilitating interactions with ubiquitin domain-containing proteins and activates the NF-kappa-B pathway. Monoubiquitinated on Lys-277 and Lys-309; promotes nuclear export. Linear polyubiquitinated on Lys-285; the head-to-tail polyubiquitination is mediated by the LUBAC complex. Linear polyubiquitinated on Lys-309; the head-to-tail polyubiquitination is mediated by the LUBAC complex.
    Sumoylated on Lys-277 and Lys-309 by SUMO1; the modification results in phosphorylation of Ser-85 by ATM leading to a replacement of the sumoylation by mono-ubiquitination on these residues.
  • Cellular localization

    Cytoplasm. Nucleus. Sumoylated NEMO accumulates in the nucleus in response to genotoxic stress.
  • Information by UniProt

Images

  • SDS-PAGE analysis of ab206008 (5µg) Coomassie-stained.

References

ab206008 has not yet been referenced specifically in any publications.

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