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RELA

MIM.164014

Tuesday 8 January 2008

WKP

Definition: 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 translocation and activation.

NF-κB is an essential transcription factor complex involved in all types of cellular processes, including cellular metabolism, chemotaxis, etc.

Phosphorylation and acetylation of RELA are crucial post-translational modifications required for NF-κB activation. RELA has also been shown to modulate immune responses, and activation of RELA is positively associated with multiple types of cancer.

Pathology

- C11orf95-RELA fusion gene in ependymoma

  • RELA-associated ependymoma

- RELA-ZMYND8 fusion gene in acute erythroid leukemia (23667654)

NFKB complex

NFKB1 (MIM.164011) or NFKB2 (MIM.164012) is bound to REL (MIM.164910), RELA (MIM.164014), or RELB (MIM.604758) to form the NFKB complex.

The p50 (NFKB1)/p65 (RELA) heterodimer is the most abundant form of NFKB. The NFKB complex is inhibited by I-kappa-B proteins (NFKBIA) (MIM.164008) or NFKBIB (MIM.604495), which inactivate NFKB by trapping it in the cytoplasm.

Phosphorylation of serine residues on the I-kappa-B proteins by kinases IKBKA (MIM.600664) or IKBKB (MIM.603258) marks them for destruction via the ubiquitination pathway, thereby allowing activation of the NFKB complex.

Activated NFKB complex translocates into the nucleus and binds DNA at kappa-B-binding motifs such as 5-prime GGGRNNYYCC 3-prime or 5-prime HGGARNYYCC 3-prime (where H is A, C, or T; R is an A or G purine; and Y is a C or T pyrimidine).

See also

NFKBs NFKB1 NFKB2 NFKB3
RELs REL RELA RELB

Open references

- C11orf95-RELA fusion present in a primary supratentorial ependymoma and recurrent sarcoma. Cachia D, Wani K, Penas-Prado M, Olar A, McCutcheon IE, Benjamin RS, Armstrong TS, Gilbert MR, Aldape KD.
Brain Tumor Pathol. 2015 Apr;32(2):105-11. doi : 10.1007/s10014-014-0205-1
PMID: 25388523 Free

- C11orf95-RELA fusions drive oncogenic NF-κB signalling in ependymoma.
Parker M, Mohankumar KM, Punchihewa C, Weinlich R, Dalton JD, Li Y, Lee R, Tatevossian RG, Phoenix TN, Thiruvenkatam R, White E, Tang B, Orisme W, Gupta K, Rusch M, Chen X, Li Y, Nagahawhatte P, Hedlund E, Finkelstein D, Wu G, Shurtleff S, Easton J, Boggs K, Yergeau D, Vadodaria B, Mulder HL, Becksfort J, Gupta P, Huether R, Ma J, Song G, Gajjar A, Merchant T, Boop F, Smith AA, Ding L, Lu C, Ochoa K, Zhao D, Fulton RS, Fulton LL, Mardis ER, Wilson RK, Downing JR, Green DR, Zhang J, Ellison DW, Gilbertson RJ. Nature. 2014 Feb 27;506(7489):451-5. doi : 10.1038/nature13109
PMID: 24553141 Free

- Fusion of ZMYND8 and RELA genes in acute erythroid leukemia.
Panagopoulos I, Micci F, Thorsen J, Haugom L, Buechner J, Kerndrup G, Tierens A, Zeller B, Heim S.
PLoS One. 2013 May 7;8(5):e63663. doi : 10.1371/journal.pone.0063663
PMID: 23667654 Free

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