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Last flight from JFK

Last flight from JFK. Higher glycogen level in PERK KO neonates. Hepatic microsomal glucose-6-phosphatase system and SIDS.

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Last flight from JFK

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  1. Last flight from JFK

  2. Higher glycogen level in PERK KO neonates

  3. Hepatic microsomal glucose-6-phosphatase system and SIDS Microassay techniques and monospecific antibodies were used to study the hepatic glucose-6-phosphatase system in liver samples from 55 infants who had died suddenly and unexpectedly, including 38/55 victims of sudden infant death syndrome (SIDS). Raised hepatic glycogen was found in 10/38, all of whom had a diagnosis of SIDS, Of the 10 infants with raised hepatic glycogen who had a diagnosis of SIDS, 8/10 had glucose-6-phosphatase deficiency (type 1a glycogen storage disease), and 2/10 had transport protein T2 deficiency (type 1c glycogen storage disease). Burchell A, et al, Lancet. 1989;2:291-4.

  4. Phenotype of GSD-1a mice severe hypoglycemia, growth retardation, perinatal lethality, deformity of joints, hepatomegaly, kidney enlargement, hyperlipidemia. Lei KJ et al,Nat Genet. 1996;13:203-9.

  5. G6-Pase systems Catalytic subunit inside ER Transporter subunits transport G6-P into ER

  6. First test (Add Triton X100) WT1 WT2 KO1 KO2

  7. Second Test(Without Triton X100) WT1 WT2 KO1 KO2

  8. Second test-With TritonX100 KO1 Not reliable ? WT1 WT2&KO2

  9. Increased insulin sensitivity

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