ライフサイエンスコーパス: GCH

1 GCH III is a tetramer of identical subunits; each monome

2 eted overexpression of GTP cyclohydrolase 1 (GCH), the rate limiting enzyme in BH4 synthesis, increas

3 We have studied the GTP-cyclohydrolase 1 (GCH-1) gene in 30 patients with the diagnosis of clinica

4 is formally identical to that catalyzed by a GCH II ortholog (SCO 6655) from Streptomyces coelicolor;

5 nd mapping onto the structure of the E. coli GCH II protein allowed identification of a switch residu

6 ith atypical phenylketonuria due to complete GCH-1 deficiency.

7 ing BH4 synthetic enzyme GTP cyclohydrolase (GCH) became undetectable in the sweat gland neurons duri

8 rgeted overexpression of GTP cyclohydrolase (GCH) I increased levels of the endothelial NO synthase c

9 GTP cyclohydrolase (GCH) III from Methanocaldococcus jannaschii, which catal

10 Endothelial GCH overexpression increased endothelial NO synthase cou

11 s in vascular tissue and plasma samples from GCH/ApoE-KO animals.

12 f a patient with adult giant-cell hepatitis (GCH), a rare form of hepatitis with presumed viral etiol

13 ical role(s) of the gene clusters that house GCH II homologues will be discussed.

14 d from the Genomic Comparison Hybridization (GCH) experiment and performed using the Affymetrix platf

15 enic overexpression of GTP-cyclohydrolase I (GCH), prevented hypoxia-induced pulmonary hypertension.

16 mino acid identity to GTP cyclohydrolase II (GCH II), which catalyzes the committed step in the biosy

17 l hyperplasia in experimental vein grafts in GCH/apolipoprotein E-knockout mice.

18 ion of macrophage marker CD68 mRNA levels in GCH/ApoE-KO mice.

19 ne of our DRD patients without a mutation in GCH-1 had the 3-bp deletion recently detected in DYT1, t

20 domain containing proteins, are observed in GCH III.

21 RANTES (CCL5) were significantly reduced in GCH/ApoE-KO aortic tissue.

22 ed variants (Asp-->Asn) of these residues in GCH III are less active than wild-type.

23 Interestingly, GCH-1 overexpression abrogated these detrimental effects

24 s, revealed by tracking studies in Tie2-LacZ/GCH-Tg/apolipoprotein E-knockout recipient mice or donor

25 Our data are consistent with duplication of GCH II in S. coelicolor promoting evolution of a new fun

26 We further extended our investigation of GCH-1 to the 5' and 3' regulatory regions and report the

27 d SCO 2687) produce the canonical product of GCH II, 2,5-diamino-6-ribosylamino-4(3H)-pyrimidinone 5'

28 rvival and recipient-derived repopulation of GCH transgenic ECs, revealed by tracking studies in Tie2

29 The structure of GCH III complexed with GTP solved at 2 A resolution clea

30 The structure of GCH III extends the repertoire of possible reactions wit

31 es coelicolor; however, SCO 6655, like other GCH II proteins, is a zinc-containing protein.

32 a clear set of mutational events that permit GCH II to produce either FAPy or APy.

33 olate was more pathogenic than the reference GCH-1 isolate.

34 weat glands reduce BH4 levels by suppressing GCH expression during development.

35 OCA-salt mice, but both were preserved in Tg-GCH mice despite DOCA-salt treatment.

36 othelium-specific GTPCH I overexpression (Tg-GCH).

37 gnificantly improved in DOCA-salt-treated Tg-GCH mice, in parallel with reduced O2(-) levels.

38 fects were prevented in DOCA-salt-treated Tg-GCH mice.

39 ls but was preserved in DOCA-salt-treated Tg-GCH mice.

40 These data also indicate that GCH expression, which is often coordinately regulated wi

41 solved at 2 A resolution clearly shows that GCH III adopts a distinct fold that is closely related t

42 metal ions in the active site suggests that GCH III utilizes two metal ion catalysis.

43 to be an important causative factor for the GCH-1 mutations in DRD.

44 ced vein graft atherosclerosis in transgenic GCH/ApoE-KO mice compared to ApoE-KO controls.

45 dothelial BH4 in hph-1 mice by crossing with GCH transgenic mice rescued pulmonary hypertension induc

46 In contrast, livers of patients with GCH display strong GP73 immunoreactivity in multinucleat