ライフサイエンスコーパス: LCAD

1 LCAD -/- mice that reached birth appeared normal, but ha

2 LCAD is deacetylated in wild-type mice under fasted cond

3 LCAD(-/-) mice demonstrated reduced pulmonary compliance

4 LCAD(-/-) surfactant demonstrated functional abnormaliti

5 hy, and sudden death was observed in 4 of 75 LCAD -/- mice.

6 Approximately 10% of adult LCAD -/- males developed cardiomyopathy, and sudden deat

7 e the crucial roles of mitochondrial FAO and LCAD in vivo.

8 dentify the fatty acid oxidation pathway and LCAD in particular as factors contributing to the pathop

9 Matings between LCAD +/- mice yielded an abnormally low number of LCAD +

10 ng from sires and dams heterozygous for both LCAD and VLCAD mutations.

11 use it is less efficiently dehydrogenated by LCAD than is its cis isomer and that the accumulation of

12 Progeny with the combined LCAD(+/+)//VLCAD(+/-) genotype were over-represented in

13 In contrast, no live mice with a compound LCAD(-/-)//VLCAD(-/-) genotype were detected.

14 CoA synthase, ACO, CYP4A3) and other (CPT1, LCAD) genes of mitochondrial and extramitochondrial LCFA

15 cal phenotypes of mice with LCAD deficiency (LCAD(-/-)) and wild-type mice.

16 at long-chain acyl coenzyme A dehydrogenase (LCAD) is hyperacetylated at lysine 42 in the absence of

17 study of long-chain acyl-CoA dehydrogenase (LCAD) and very long-chain acyl-CoA dehydrogenase reveale

18 Long-chain acyl-CoA dehydrogenase (LCAD) catalyzes the initial step in mitochondrial fatty

19 nted with long-chain acyl-CoA dehydrogenase (LCAD) deficiency.

20 Long-chain acyl-CoA dehydrogenase (LCAD) is a key mitochondrial fatty acid oxidation enzyme

21 Long-chain acyl-CoA dehydrogenase (LCAD) is a mitochondrial fatty acid oxidation enzyme who

22 ciency of long-chain acyl-CoA dehydrogenase (LCAD), a key enzyme in mitochondrial fatty acid oxidatio

23 in human long chain acyl-CoA dehydrogenase (LCAD), has been suggested to affect substrate chain leng

24 on enzyme long-chain acyl-CoA dehydrogenase (LCAD).

25 background with a more severe defect in FAO (LCAD(-/-); VLCAD(+/-)) in addition to a validated mouse

26 Large aggregate surfactant isolated from LCAD(-/-) mouse lavage fluid had significantly reduced p

27 y surfactant, was significantly increased in LCAD(-/-) lavage fluid, suggesting increased epithelial

28 ed, accumulate at the air-fluid interface in LCAD(-/-) lungs.

29 ) and C(14) acylcarnitines were prominent in LCAD(-/-) mice.

30 Lung fatty acid oxidation was reduced in LCAD(-/-) mice.

31 We previously demonstrated increased LCAD lysine acetylation in SIRT3 knockout mice concomita

32 udden unexplained infant death where no lung LCAD antigen was detectable.

33 /-)) in addition to a validated mouse model (LCAD(-/-); VLCAD(+/+)) and compared them with wild-type

34 n vitro and in vivo; and hyperacetylation of LCAD reduces its enzymatic activity.

35 This study demonstrates localization of LCAD to human alveolar type II pneumocytes, which synthe

36 We produced a mouse model of LCAD deficiency with severely impaired FAO.

37 +/- mice yielded an abnormally low number of LCAD +/- and -/- offspring, indicating frequent gestatio

38 The physiological role of LCAD and the fatty acid oxidation pathway in lung was su

39 s-tetradecenoyl-CoA is a poorer substrate of LCAD than is 5-cis-tetradecenoyl-CoA, while both unsatur

40 residue at a position homologous to that of LCAD, was also synthesized and purified.

41 Treatment of LCAD(-/-) mice with mildronate, a drug that inhibits car

42 To study the effects of acetylation on LCAD and determine sirtuin 3 (SIRT3) target sites, we ch

43 sites, we chemically acetylated recombinant LCAD.

44 SIRT3 knockout mice concomitant with reduced LCAD activity and reduced fatty acid oxidation.

45 Here, we show that LCAD knockout mice develop hepatic steatosis, which is a

46 degrees C drop in body temperature in unfed LCAD(-/-); VLCAD(+/+) mice compared with WT body tempera

47 thway in lung was subsequently studied using LCAD knock-out mice.

48 and 64% of wild-type, respectively), whereas LCAD(-/-) mice showed a more profoundly reduced activity

49 ogic and biochemical phenotypes of mice with LCAD deficiency (LCAD(-/-)) and wild-type mice.