ライフサイエンスコーパス: fumarylacetoacetate

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1 ure and fatal metabolic liver disease due to fumarylacetoacetate dehydrolase (Fah) deficiency, hiHeps

2 , which converts maleylacetoacetate (MAA) to fumarylacetoacetate (FAA).

3 tary tyrosinemia type 1 have a deficiency of fumarylacetoacetate hydrolase (FAH) and develop progress

4 tyrosine catabolism caused by deficiency of fumarylacetoacetate hydrolase (FAH) and homogentisic aci

5 Fumarylacetoacetate hydrolase (FAH) catalyzes the hydrol

6 ss of the remaining Hgd allele protects from fumarylacetoacetate hydrolase (Fah) deficiency, a geneti

7 es (BMHs) can cure the genetic liver disease fumarylacetoacetate hydrolase (Fah) deficiency.

8 Transplantation into fumarylacetoacetate hydrolase (Fah) deficient mice was u

9 Mice doubly mutant for MAAI and fumarylacetoacetate hydrolase (FAH) died rapidly on a no

10 Fumarylacetoacetate hydrolase (FAH) domain-containing pr

11 tic genotoxicity in vivo, we transferred the fumarylacetoacetate hydrolase (FAH) gene by LV vectors i

12 associated with point mutations in the human fumarylacetoacetate hydrolase (FAH) gene that disrupt ty

13 tis B virus X (HBx) gene, into the livers of fumarylacetoacetate hydrolase (Fah) mutant mice via hydr

14 analysis identified 4-OD as a member of the fumarylacetoacetate hydrolase (FAH) superfamily and impl

15 essive liver disease caused by deficiency of fumarylacetoacetate hydrolase (FAH), to determine whethe

16 stem, we generated severely immunodeficient, fumarylacetoacetate hydrolase (Fah)-deficient mice.

17 inemia and show that the treatment generated fumarylacetoacetate hydrolase (Fah)-positive hepatocytes

18 and is caused by a deficiency in the enzyme fumarylacetoacetate hydrolase (FAH).

19 ns affecting the last enzyme in the pathway, fumarylacetoacetate hydrolase (FAH).

20 cells into syngeneic recipients deficient in fumarylacetoacetate hydrolase and manifesting tyrosinemi

21 the chemistries of ureidoglycolate lyase and fumarylacetoacetate hydrolase catalysis.

22 ocytes can repopulate the liver of mice with fumarylacetoacetate hydrolase deficiency and correct the

23 serial transplantation of hepatocytes in the fumarylacetoacetate hydrolase deficiency murine model of

24 s needed for liver regeneration in mice with fumarylacetoacetate hydrolase deficiency.

25 In this study, we investigated whether fumarylacetoacetate hydrolase deficient (FAH(-/-)) pigs,

26 In healthy fumarylacetoacetate hydrolase deficient mice (Fah(-/-)),

27 e exhibits a high level of similarity to the fumarylacetoacetate hydrolase family of proteins.

28 osinaemia type I, mice with mutations in the fumarylacetoacetate hydrolase gene (Fah-/-) regain norma

29 Here, we used the fumarylacetoacetate hydrolase knockout mouse to determin

30 aperitoneal injection into 8- to 12-week-old fumarylacetoacetate hydrolase mice (Fah(-/-)), a model o

31 omic sequence for repairing the mutated Fah (fumarylacetoacetate hydrolase) gene.

32 al recessive disease caused by deficiency in fumarylacetoacetate hydrolase, the last enzyme in the ty

33 ion of human hepatocytes in immunodeficient, fumarylacetoacetate hydrolase-deficient (fah(-/-)) mice.

34 In addition, 28 of the fumarylacetoacetate hydrolase-deficient mice were transp

35 mice long after death and transplanted into fumarylacetoacetate hydrolase-deficient mice, a model fo

36 iparum LS in vivo: the immunocompromised and fumarylacetoacetate hydrolase-deficient mouse (Fah-/-, R

37 ocytes expressing dipeptidyl peptidase IV or fumarylacetoacetate hydrolase.

38 yrosinemia type 1 is caused by deficiency of fumarylacetoacetate hydrolase.

39 trans isomerisation of maleylacetoacetate to fumarylacetoacetate is the penultimate step in the tyros

40 e enzymatic defect impairs the conversion of fumarylacetoacetate to fumarate, causing accumulation of

41 drolytic cleavage of a carbon-carbon bond in fumarylacetoacetate to yield fumarate and acetoacetate a

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