IκBα
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IκBα (nuclear factor of kappa light polypeptide gene enhancer in B-cells inhibitor, alpha) is one member of a family of cellular proteins that function to inhibit the
NF-κB Nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) is a protein complex that controls transcription of DNA, cytokine production and cell survival. NF-κB is found in almost all animal cell types and is involved in cellular ...
transcription factor In molecular biology, a transcription factor (TF) (or sequence-specific DNA-binding factor) is a protein that controls the rate of transcription of genetic information from DNA to messenger RNA, by binding to a specific DNA sequence. The fu ...
. IκBα inhibits NF-κB by masking the
nuclear localization signal A nuclear localization signal ''or'' sequence (NLS) is an amino acid sequence that 'tags' a protein for import into the cell nucleus by nuclear transport. Typically, this signal consists of one or more short sequences of positively charged lysines o ...
s (NLS) of NF-κB proteins and keeping them sequestered in an inactive state in the cytoplasm. In addition, IκBα blocks the ability of NF-κB transcription factors to bind to DNA, which is required for NF-κB's proper functioning.


Disease linkage

The gene encoding the IκBα protein is mutated in some
Hodgkin's lymphoma Hodgkin lymphoma (HL) is a type of lymphoma, in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the patient's lymph nodes. The condition wa ...
cells; such mutations inactivate the IκBα protein, thus causing NF-κB to be chronically active in the lymphoma tumor cells and this activity contributes to the malignant state of these tumor cells.


Interactions

IκBα has been shown to
interact Advocates for Informed Choice, dba interACT or interACT Advocates for Intersex Youth, is a 501(c)(3) nonprofit organization using innovative strategies to advocate for the legal and human rights of children with intersex traits. The organizati ...
with: * BTRC, *
C22orf25 Transport and golgi organization 2 homolog (TANGO2) also known as chromosome 22 open reading frame 25 (C22orf25) is a protein that in humans is encoded by the TANGO2 gene. The function of C22orf25 is not currently known. It is characterized by ...
, *
CHUK Inhibitor of nuclear factor kappa-B kinase subunit alpha (IKK-α) also known as IKK1 or conserved helix-loop-helix ubiquitous kinase (CHUK) is a protein kinase that in humans is encoded by the ''CHUK'' gene. IKK-α is part of the IκB kinase ...
, *
DYNLL1 Dynein light chain 1, cytoplasmic is a protein that in humans is encoded by the ''DYNLL1'' gene. Function Cytoplasmic dyneins are large enzyme complexes with a molecular mass of about 1,200 kD. They contain two force-producing heads formed prim ...
, *
G3BP2 Ras GTPase-activating protein-binding protein 2 is an enzyme that in humans is encoded by the ''G3BP2'' gene. Interactions G3BP2 has been shown to interact with IκBα IκBα (nuclear factor of kappa light polypeptide gene enhancer in B-cel ...
, *
Heterogeneous nuclear ribonucleoprotein A1 Heterogeneous nuclear ribonucleoprotein A1 is a protein that in humans is encoded by the ''HNRNPA1'' gene. Mutations in hnRNP A1 are causative of amyotrophic lateral sclerosis and the syndrome multisystem proteinopathy. Function This gene ...
, *
IKK2 IKK-β also known as inhibitor of nuclear factor kappa-B kinase subunit beta is a protein that in humans is encoded by the ''IKBKB'' (inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase beta) gene. Function IKK-β is an enzy ...
, *
NFKB1 Nuclear factor NF-kappa-B p105 subunit is a protein that in humans is encoded by the ''NFKB1'' gene. This gene encodes a 105 kD protein which can undergo cotranslational processing by the 26S proteasome to produce a 50 kD protein. The 105 kD prot ...
, *
P53 p53, also known as Tumor protein P53, cellular tumor antigen p53 (UniProt name), or transformation-related protein 53 (TRP53) is a regulatory protein that is often mutated in human cancers. The p53 proteins (originally thought to be, and often s ...
, *
RELA Transcription factor p65 also known as nuclear factor NF-kappa-B p65 subunit is a protein that in humans is encoded by the ''RELA'' gene. RELA, also known as p65, is a REL-associated protein involved in NF-κB heterodimer formation, nuclear tran ...
, *
RPS6KA1 Ribosomal protein S6 kinase alpha-1 is an enzyme that in humans is encoded by the ''RPS6KA1'' gene. Function This gene encodes a member of the RSK (ribosomal S6 kinase) family of serine/threonine kinases. This kinase contains 2 nonidentical k ...
, *
SUMO4 Small ubiquitin-related modifier 4 is a protein that in humans is encoded by the ''SUMO4'' gene. Function This gene is a member of the SUMO gene family. This family of genes encode small ubiquitin-related modifiers that are attached to proteins ...
, and *
Valosin-containing protein Valosin-containing protein (VCP) or transitional endoplasmic reticulum ATPase (TER ATPase) also known as p97 in mammals and CDC48 in ''S. cerevisiae,'' is an enzyme that in humans is encoded by the ''VCP'' gene. The TER ATPase is an ATPase enzyme ...
.


References


Further reading

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External links


OMIM entries on Ectodermal Dysplasia, Anhidrotic, with T-cell Immunodeficiency
* * * {{DEFAULTSORT:Ikappabetaalpha Transcription factors