ID:MLXPL_HUMAN DESCRIPTION: RecName: Full=Carbohydrate-responsive element-binding protein; Short=ChREBP; AltName: Full=Class D basic helix-loop-helix protein 14; Short=bHLHd14; AltName: Full=MLX interactor; AltName: Full=MLX-interacting protein-like; AltName: Full=WS basic-helix-loop-helix leucine zipper protein; Short=WS-bHLH; AltName: Full=Williams-Beuren syndrome chromosomal region 14 protein; FUNCTION: Transcriptional repressor. Binds to the canonical and non-canonical E box sequences 5'-CACGTG-3' (By similarity). SUBUNIT: Binds DNA as a heterodimer with TCFL4/MLX. SUBCELLULAR LOCATION: Nucleus. TISSUE SPECIFICITY: Expressed in liver, heart, kidney, cerebellum and intestinal tissues. PTM: Phosphorylation at Ser-556 by AMPK inactivates the DNA- binding activity (By similarity). DISEASE: Note=WBSCR14 is located in the Williams-Beuren syndrome (WBS) critical region. WBS results from a hemizygous deletion of several genes on chromosome 7q11.23, thought to arise as a consequence of unequal crossing over between highly homologous low-copy repeat sequences flanking the deleted region. Haploinsufficiency of WBSCR14 may be the cause of certain cardiovascular and musculo-skeletal abnormalities observed in the disease. SIMILARITY: Contains 1 bHLH (basic helix-loop-helix) domain.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q9NP71
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
AF056184 - Homo sapiens WS basic-helix-loop-helix leucine zipper protein mRNA, complete cds. AF245470 - Homo sapiens Mlx interactor alpha (MIO) mRNA, complete cds, alternatively spliced. AF245471 - Homo sapiens Mlx interactor beta (MIO) mRNA, complete cds, alternatively spliced. AF245472 - Homo sapiens Mlx interactor gamma (MIO) mRNA, complete cds, alternatively spliced. AF245473 - Homo sapiens Mlx interactor delta (MIO) mRNA, complete cds, alternatively spliced. AF245474 - Homo sapiens Mlx interactor epsilon (MIO) mRNA, complete cds, alternatively spliced. JC178373 - Sequence 4 from Patent WO2013112458. BC012925 - Homo sapiens MLX interacting protein-like, mRNA (cDNA clone MGC:9556 IMAGE:3866131), complete cds. AF156603 - Homo sapiens putative hepatic transcription factor (WBSCR14) mRNA, complete cds. JD134428 - Sequence 115452 from Patent EP1572962. JD373321 - Sequence 354345 from Patent EP1572962. JD134427 - Sequence 115451 from Patent EP1572962. JD554863 - Sequence 535887 from Patent EP1572962. JD423683 - Sequence 404707 from Patent EP1572962. JD153554 - Sequence 134578 from Patent EP1572962. JD103309 - Sequence 84333 from Patent EP1572962. JD273333 - Sequence 254357 from Patent EP1572962. JD471590 - Sequence 452614 from Patent EP1572962. JD363081 - Sequence 344105 from Patent EP1572962. JD046098 - Sequence 27122 from Patent EP1572962. JD363233 - Sequence 344257 from Patent EP1572962. JD512219 - Sequence 493243 from Patent EP1572962. JD235431 - Sequence 216455 from Patent EP1572962. JD126136 - Sequence 107160 from Patent EP1572962. JD099570 - Sequence 80594 from Patent EP1572962. JD271436 - Sequence 252460 from Patent EP1572962. JD209304 - Sequence 190328 from Patent EP1572962. JD563763 - Sequence 544787 from Patent EP1572962. AB527610 - Synthetic construct DNA, clone: pF1KB0430, Homo sapiens MLXIPL gene for MLX interacting protein-like, without stop codon, in Flexi system. JD133879 - Sequence 114903 from Patent EP1572962. JD103295 - Sequence 84319 from Patent EP1572962. JD398209 - Sequence 379233 from Patent EP1572962. JD450434 - Sequence 431458 from Patent EP1572962. JD239741 - Sequence 220765 from Patent EP1572962. JD098647 - Sequence 79671 from Patent EP1572962. JD465038 - Sequence 446062 from Patent EP1572962. AK055029 - Homo sapiens cDNA FLJ30467 fis, clone BRAWH1000002, highly similar to Homo sapiens putative hepatic transcription factor (WBSCR14) mRNA. JD119070 - Sequence 100094 from Patent EP1572962.
Biochemical and Signaling Pathways
BioCarta from NCI Cancer Genome Anatomy Project h_chrebpPathway - ChREBP regulation by carbohydrates and cAMP
Reactome (by CSHL, EBI, and GO)
Protein Q9NP71 (Reactome details) participates in the following event(s):
R-HSA-163666 Formation of ChREBP:MLX heterodimer R-HSA-163672 Phosphorylation of ChREBP at Thr(666) by PKA R-HSA-163691 Phosphorylation of ChREBP at Serine 568 by AMPK R-HSA-163688 Dephosphorylation of pChREBP (Thr 666) by PP2A R-HSA-164056 Dephosphorylation of pChREBP (Ser 568) by PP2A R-HSA-164423 PhosphoChREBP (Thr 666) is exported to cytosol R-HSA-163670 Nuclear transport of pChREBP (Thr 666) protein R-HSA-163689 Dephosphorylation of pChREBP (Ser 196) by PP2A R-HSA-163676 Phosphorylation of pChREBP (Thr 666) at Ser(196) by PKA R-HSA-163765 ChREBP activates metabolic gene expression R-HSA-163358 PKA-mediated phosphorylation of key metabolic factors R-HSA-163680 AMPK inhibits chREBP transcriptional activation activity R-HSA-163767 PP2A-mediated dephosphorylation of key metabolic factors R-HSA-163685 Energy Metabolism R-HSA-1430728 Metabolism