PGC-1alpha: looking behind the sweet treat for porphyria.
Porphyrias are a group of genetic disorders caused by mutations in enzymes of the heme biosynthesis pathway. Acute attacks of porphyria, reputedly the disease that incapacitated the British sovereign King George III (see this Cell cover), are precipitated by fasting and are treated by infusing heme or glucose, although the underlying molecular mechanisms remain unclear. In this issue of Cell, reveal that a transcriptional coactivator called PGC-1alpha is a key player in both induction of porphyria by fasting and amelioration of the symptoms by glucose treatment.
Li
Dangsheng
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Cell Press, Cambridge, Massachusetts 02138, USA.
D000624:5-Aminolevulinate Synthetase / Q000378:metabolism; D000818:Animals; D004789:Enzyme Activation / Q000502:physiology; D005215:Fasting / Q000009:adverse effects / Q000502:physiology*; D005947:Glucose / Q000378:metabolism*; D006360:Heat-Shock Proteins / Q000378:metabolism*; D006418:Heme / Q000096:biosynthesis*; D006801:Humans; D000071248:Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; D011164:Porphyrias / Q000209:etiology / Q000235:genetics / Q000378:metabolism*; D014157:Transcription Factors / Q000378:metabolism*; D015533:Transcriptional Activation / Q000502:physiology
D006360:Heat-Shock Proteins; C486497:PPARGC1A protein, human; D000071248:Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; D014157:Transcription Factors; D006418:Heme; D000624:5-Aminolevulinate Synthetase; C480079:ALAS2 protein, human; D005947:Glucose