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குளுக்கோஸ் 6-பாஸ்படேஸ் குறைபாடு உருவாக்குவதுஅ. காலக்டோசீமியா ஆ. அல்பினிசம்இ. வான்-கிரீக்ஸ் நோய் ஈ. ஹீமோபீலியா |
Answer» GSDI is caused by a dysfunction in the G6P system, a KEY step in the regulation of glycemia. The deficit concerns the catalytic subunit G6P-alpha (TYPE Ia) which is restricted to expression in the liver, kidney and intestine, or the ubiquitously expressed G6P transporter (type Ib)Liver cells express the transmembrane ENZYME glucose 6-phosphatase in the endoplasmic reticulum. ... Muscle cells lack this enzyme, so myofibers use glucose 6-phosphate in their own metabolic pathways such as glycolysis. Importantly, this prevents myocytes from RELEASING glycogen stores they have obtained into the blood. |
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