Chuy»ƒn h³a protein 2

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    27-Jul-2015
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Transcript of Chuy»ƒn h³a protein 2

1. Trao i trc tuyn ti: http://www.mientayvn.com/Y_online.html 2. CHUYN HO PROTEIN 3. THOI HA -COOH CA CC ACID AMIN L phn ng kh carboxyl ca aa to amin tng ng Phc hp enzym decarboxylase v coenzym vitamin B6 R CH COOH NH2 R CH2 NH2 + CO2 decarboxylase Acid amin Amin tng ng Coenzym pyridoxal phosphat 4. THOI HO NHM -COOH Histidine CO2 Histamine Histidine decarboxylase Glutamate CO2 -Aminobutyrate Glutamate decarboxylase Lysine CO2 Cadaverine Lysine decarboxylase Tyrosine CO2 Tyramine Tyrosine decarboxylase 5. aldehyt Diamin Amin + NH3 kh amin OXHkh amin OXH + 1/2 O2 Monoaminoxydaz (MAO) Diaminoxydaz CO2 H2O R THOI HO NHM -COOH 6. Lysine decarboxylase Tyrosine decarboxylase Tyrosine Tyramine Cadaverine Lysine 7. THOI HO SN CARBON 8. NH3 keto acid Amin ho cc keto acid to cc acid amin Ala Gln Urea To cht bo hay glucose Amin ho hay chuyn amin to acid amin khng cn thit Vo chu trnh Krebs to CO2 v H2O Acid amin 9. TNG HP ACID AMIN 10. Qu trnh tng hp acid amin L qu trnh gn nhm amin vo sn carbon tng ng Nhm amin: t thc n (VSV, thc vt, ng vt). Sau , c s dng to acid amin mi di dng nhm amin ca glutamine hoc glutamate Khung carbon : t cc sn phm chuyn ha trung gian 11. ACID AMIN CN THIT V KHNG CN THIT Acid amin cn thit Acid amin khng cn thit C th ng vt c v khng tng hp c, cn phi ly t thc n Arginine Histidine Isoleucine Leucine Lysine Methionine Phenylalanine Threonine Tryptophan Valine C th ng vt c v tng hp c Glycine Alanine Cystein Acid glutamic Glutamine Acid aspartic Asparagine Tyrosine Proline Serine Arg c tng hp trong chu trnh UREA, nhng tc rt thp khng p ng nhu cu pht trin tr em 12. Khi qut tng hp acid amin Tng hp ca hu ht acid amin bt u t cc sn phm trung gian ca TCA Ty theo cht ban u, tng hp acid amin c phn thnh cc nhm khc nhau 13. Khi qut tng hp acid amin 14. Phn ng amin ho 15. Phn ng chuyn amin 16. AlaninePyruvate Tng hp ALANINE, ASPARTATE, ASPARAGINE t PYRUVATE v OXALOACETATE 17. Tng hp alanine 18. Tng hp aspartate, asparagine 19. Tng hp glutamate, glutamineTng hp Glutamate, Glutamine 20. Tng hp SERINE, GLYCINE, CYSTEINE t 3-PHOSPHOGLYCERATE t 21. Tng hp serine 22. Tng hp glycine Tng hp cystein serine + THF --> glycine + N5,N10 -methylene-THF (enzyme: serine hydroxymethyltransferase) N5,N10 -methylene-THF + CO2 + NH4 + --> glycine (enzyme: glycine synthase; cn NADH) Homocysteine l sn phm thoi ho ca methionine serine + homocysteine -> cystathionine + H2O cystathionine + H2O --> -ketobutyrate + cysteine + NH3 THF: tetrahydrofolate 23. Tng hp tyrosine 24. Tng hp proline Tng hp Proline 25. CHUYN HO CHUYN BIT CA MT S ACID AMIN 26. ACID AMIN TO NG ACID AMIN TO CETON 27. GLYCIN v SERIN gan: CH2OH H2N CH COOH CHO H2N CH COOH H H2N CH COOH + HCOOH Serin Glycin Acid formic - H2 + H2O Trong c th: glycin b kh amin oxy ha bi enzym dehydrase NH2 H CH COOH O H CH COOH O OH CH COOH H COOH H2O + CO2 CO2 - NH3 +1/2O2 O Glycin Acid glyoxylic Acid oxalic Trong chuyn ha, glycin tham gia cc qu trnh sinh tng hp lin quan n acid mt (acid glycolic), creatinin, heme, base purin 28. GLUTAMATE (1) PLP : Pyridoxal phosphate Tng hp GABA : -aminobutyric acid l cht c ch dn truyn thn kinh chnh ca h TKTW Thiu GABA mt kim ch, ng kinh C ch ca GABA : tnh thm ca knh Cl- BENZODIAZEPINES ( ng kinh) : tnh thm mng i vi Clo tc dng ca GABA 29. To Glutathione ti gan Glutamate (2) 30. Glutathion tham gia cc qu trnh oxy ho sinh hc: 31. CHUYN HO HISTIDINE 32. Chuyn ho HISTIDINE Tng hp Histamin: Histamin c phng thch p ng vi mt tnh trng d ng Histamin gy dn mch, co c trn (th th H1 ph qun, rut), tng tnh thm thnh mch,kch thch tit acid d dy (th th H2) 33. CHUYN HO TRYPTOPHAN Acid nicotinic = Niacin = Vitamin B3: tin cht ca NAD v NADP (coenzym ca cc phn ng oxy ha kh) 34. Chuyn ho TRYPTOPHAN Tng hp Serotonin: Xy ra h TKT, ng tiu ha Chc nng dn truyn TK 35. Chuyn ho TRYPTOPHAN Tng hp Melatonin: c tng hp TB tuyn yn Tng cng gic ng do c ch s tng hp v bi tit Dopamin v GABA TB no vo ban m 36. S TNG QUT CHUYN HO PHENYLALANINE V TYROSINE CHUYN HO PHENYLALANINE 37. TNG HP MELANINE 38. THOI HO TYROSINE Homogentisat = Alcapton 39. HORMONE TUYN GIP 40. TNG HP CATECHOLAMINE COMT: catechol-O-methyltransferase MAO: monoamine oxidase 41. Tng hp NO t Arginine 42. CHUYN HO CREATIN THN - GAN C 43. Tng hp Creatin v Creatinin 44. Creatin Do gan to ra 98% creatin v c (20-30% creatin, 70- 80% creatin P) L ngun d tr nng lng ca c To creatin P trong ty th/c Phng thch nng lng to ATP trong bo cht/c 45. Creatinin Sn phm kh nc (khng cn enzyme) ca creatin 1,5% creatin chuyn thnh creatinin/ngy (1,8g). Lng creatinin ph thuc vo lng c (tui, gii) nh gi chc nng lc ca thn 46. To mui mt: To porphyrin: To purin, pyrimidin: 47. To acid nicotinic (vit PP) t Tryptophan To MET di dng hot ha giu nng lng, d dng cho nhm CH3 trong tng hp cht (cystein) hay methyl ha nhiu cht quan trng nh: 48. BNH L DI TRUYN LIN QUAN N CHUYN HA ACID AMIN Phenylketonuria (Phenyl ceton niu) Suy tuyn gip bm sinh MAPLE SYRUS URINE DISEASE Alcaptonuria (Alcapton niu) Bch tng (Albinism) 49. BNH L DI TRUYN LIN QUAN N CHUYN HA ACID AMIN 50. Phenylketonuria (Phenyl ceton niu) 51. Phenylketonuria (Phenyl ceton niu) Nguyn nhn: - Khim khuyt di truyn v to PAH (NST 12q22-24). - Khim khuyt v enzyme ca tetrahydrobiopterin. Tyr tr thnh aa cn thit Phenylalanin tng cao trong mu Phenyllactic v phenylacetic tng cao trong nc tiu Tn sut:1:20.000 tr s sinh Nu khng c pht hin v ngn nga sm, tr s b chm pht trin v mt tr tu Pht hin sm, tr c iu tr bng ch n hn ch Phe s pht trin bnh thng. 52. Suy tuyn gip bm sinh Trong suy gip bm sinh (hypothyroidism), tuyn gip khng sn xut hormon gip (v d T4). TSH cao do tuyn gip kch thch v thiu feedback t T4 v T3 (v nng thp). 53. Suy tuyn gip bm sinh - Ph bin l chm pht trin tr tu. Tuy nhin, khng triu chng lc sinh. Nu khng c pht hin sm s dn ti chng ngu n (cretinism), chm pht trin c v tr tu v th lc. - Tn sut chung 1:3500 - Sng lc bng TSH hoc T4 t mu mu kh, ly vo ngy th 3-5 - Ph bin l sng lc TSH, khng nh bng T4 - Gi tr cut-off 15-20mU/l TSH /mu ton phn. - Nu c iu tr sm, tin lng tt iu tr cng sm cng tt. iu tr hormon tuyn gip, T4 hay T3, r - Theo di quan trng nht l trong nm u 54. MAPLE SYRUS URINE DISEASE 1/760 nhng gia nh Mennonites. Thiu enzym BCKAD (Branched-chain ketoacid dehydrogenase) . Sau sinh c nhng bt thng v thn kinh nh co git, hn m, c th t vong. Tng leucin, isoleucin v valin (aa c nhnh) trong huyt tng v trong nc tiu. Xut hin cc dng oxo-acid bt thng/nc tiu (mi l) iu tr: Nhanh chng lm gim leucin v cc BCAA khc. Cung cp nng lng, cc thc phm dinh dng khng cha BCAA cho tr. 55. Alcaptonuria (Alcapton niu) Ri lon di truyn bm sinh do thiu homogentisate oxygenase Tch t homogentisate, bi tit ra nc tiu, c mu en do homogentisate b oxy ha Tch t acid homogentisic trong m lin kt, gy vim khp thoi ha Tn sut : 1:200.000 56. MAPLE SYRUS URINE DISEASE 57. Protein mu Nng protein ton phn/huyt thanh: 65-85 g/l 58. 1. Protein mu gim : - Gim cung cp: Ri lon tiu ha, RL hp thu... - Gim tng hp: Bnh l v GAN - Mt protein: qua ng niu, tiu ha, mt mu.. -Tng phn hy: Tiu ng, cng gip trng 2. Tng protein/mu : - C c mu - Protein bt thng: Bence Jone protein (Kahler) 59. Protein niu Bnh thng: < 150mg/24h: protein NT (-), vt Bnh l: >>150mg/24h, ko di Tng thm tch protein ca mng cu thn, c th do: 1. Bnh l thn: Vim cu thn, hc thn h 2. Xut hin protein bt thng, TLPT thp Tng khuch tn protein do gim tc dng mu ti thn: Suy tim, tng huyt p, ng lu Ri lan ti hp thu ng thn (him) Tn thng ng tit niu