Shaanxi Bloom Tech Co., Ltd. yog ib qho ntawm cov neeg tsim khoom thiab cov chaw muag khoom ntawm sapropterin hydrochloride CAS 69056-38-8 nyob rau hauv Suav teb. Txais tos rau lag luam wholesale mag zoo sapropterin hydrochloride CAS 69056-38-8 Kev muag khoom ntawm no los ntawm peb lub hoobkas. Cov kev pabcuam zoo thiab tus nqi tsim nyog muaj.
Sapropterin hydrochloride,Lub npe tshuaj lom neeg yog (6R) -5,6,8-tetrahydrobiopterin dihydrothide, uas zoo li cov hmoov nplej dawb thiab yog ib qho tseem ceeb organic thiab yog kev tshawb fawb txog kev kho mob thiab kho mob. Nws muaj qee yam toxicity, nrog ib qho LD50 (ntawm kev tswj hwm qhov ncauj kom nas) ntawm 1G / kg. Yog li ntawd, thaum siv thiab los nkag rau hauv kev sib txuas no, nws yog qhov tsim nyog kom ua raws cov txheej txheem kev nyab xeeb, coj cov khoom tiv thaiv uas tsim nyog, thiab tsis txhob sib cuag nrog tawv nqaij thiab qhov muag. Nws yog lub ntuj cofactor rau cov enzymes tseem ceeb xws li Phenyylalanine Hydroxylase (PACH) Hydroxylase (Tyrcean Hydroxylase (Tphide Oxide Synthease (Nos). Cov txheej txheem sib txuam no tau cuam tshuam nrog ntau cov txheej txheem metabolic hauv cov kab, xws li amino acid metabolism, neurotransmitter synthesis, thiab vasodilation synthesis, thiab vasodilation Raws li Cofactor, nws tuaj yeem tswj cov qauv ntawm cov enzymes, txhawb kev enzymes substrate khi, thiab ua kom nrawm nrawm rau cov kev ua kom zoo nkauj. Qhov no yog qhov tseem ceeb rau kev tswj hwm kev ua haujlwm ntawm lub cev thiab cov metabolic tshuav hauv cov kab mob.
CAS TSIS MUAJ.: 69056-3-8-8, molecular tus qauv: C9H17Cl2nCl2n5o3, molecular hnyav: 314.17, einecs tsis.: 663-669-3
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Puab |
Dawb rau ntawm cov hmoov dawb dawb |
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Xim |
tsis muaj kob rau me ntsis daj |
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soluble |
19.60 - 20.40 mg / ml |
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PH |
5-7 (10G / L, H2O, 20 degree) |
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Ceev |
1.43 (kwv yees) |
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Kev Cia |
- 20℃c |
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Kev Sib Hloov Tshwj Xeeb |
D 25 - 6.81℃(c=0.665} hauv 0.1 m hcl) |
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Melting taw tes |
245-246 Qib (Dec) |
White powder, GC>99.8%, Sab saum toj zoo rau Nyiv Qiv Pargaceutical Compaceutical Compancess.ReFence Nqe rau Qauv: 1G, $ 4G, $ 430 / g.
Sapropterin hydrochloride, Tseem hu ua Tetrahydrobiopterin hydrochloride (BH4 HCl), nws plays ib lub luag haujlwm tseem ceeb hauv Biochemistry. Raws li ib tug cofactor, nws plays lub luag haujlwm tseem ceeb hauv ntau cov amino acid metabolism, neurotransmitter synthesis, thiab nitric oxide (tsis muaj) ntau lawm.
Raws li ib tug cofactor ntawm enzymes
Ntuj cafactors ntawm ntau cov enzymes uas ua cov haujlwm metabolic tseem ceeb hauv cov kab mob.
Phenylalanine Hydroxylase (PAH)
(1) Ua haujlwm: PAH yog tus tseem ceeb enzyme nyob rau hauv lub phenylanine pathwocolic, lub luag hauj lwm rau kev hloov Penyylalanine rau hauv Tyrosine. This conversion is crucial for preventing the accumulation of phenylalanine and its metabolites, as these metabolites may cause damage to the nervous system.
(2) Lub tshuab ua haujlwm ntawm kev nqis tes ua: raws li ib tug cofactor ntawm pah, nws txhim kho kev ua tau zoo ntawm Pah los ntawm cov enzyme cov enzyme kev ua haujlwm thiab txhawb nqa subtstrate enzyme khi.
Tyrosine hydroxylase (th)
(1) Ua haujlwm: Th yog ib qho tseem ceeb enzyme hauv biosynthesis ntawm dopamine thiab norepinephrine, lub luag hauj lwm rau hloov tyrosine rau dopa (l {{1} dopa). Cov neeg neurotransmitters ua haujlwm tseem ceeb hauv kev tswj hwm kev xav, kev txav mus los, kev ua haujlwm, thiab tus nqi zog mencition, thiab tus nqi zog mechanisms.
(2) Kev Ua Haujlwm: Zoo ib yam li PAH, nws tseem yog COFORROR ntawm th, Txhawb kev enzyme catalytic kev ua ub no los ntawm kev sib txuam zoo sib xws.
Tryptophan Hydroxylase (Tph)
(1) Txoj Haujlwm: Tph yog tus tseem ceeb enzyme nyob rau hauv biosynthesis ntawm serotonin, lub luag hauj lwm rau hloov tryptophan mus rau 5-Hydroxyertophan. Serotonin ua lub luag haujlwm tseem ceeb hauv kev tswj hwm kev xav, pw tsaug zog, qab los noj mov, thiab kev nkag siab mob.
(2) Lub tshuab ntawm kev ua: raws li cofactor ntawm tph, nws tseem txhim kho cov enzyme los ntawm stabilizing nws cov qauv thiab txhawb nqa substrate enzyme khi.
NITRric Oxide Synthease (Nos)
Cov. (1) Muaj nuj nqi: Nos yog ib hom enzyme uas catchalyzes cov tshuaj tiv thaiv nruab nrab ntawm l}}} thiab l {} citrulmine. Tsis yog, raws li kev piav qhia tseem ceeb ntawm lub cev, ua haujlwm ntau lub zog ntawm lub cev hauv cov hlab plawv, hlab hlwb, thiab kev tiv thaiv kab mob.
(2) Kev Ua Haujlwm ntawm Kev Ua: Txawm hais tias tsis yog ib qho kev ua haujlwm ncaj qha ntawm Nos, nws tsis ncaj ncees rau cov metabolism ntawm nos substrates (xws li l}}}}}}}} tsis muaj kev tsim khoom.
Kho cov kab mob caj ces metabolic
Tau pom cov teebmeem tseem ceeb hauv kev kho mob muaj qee yam kab mob genabolic, tshwj xeeb yog cov muaj feem xyuam rau BH4 tsis muaj peev xwm.
Hyperphenylalaninemia (Pku)
(1) CIM: PKU yog ib tus mob caj dab ntxiv los ntawm kev sib hloov hauv PAH GENE. Qhov txo qis lossis ua tiav cov kev ua si Pah ua rau cov neeg mob lub cev ua rau ib txwm muaj, ua rau muaj kev sib tw thiab kev tsim cov tshuaj lom me me hauv lub cev.
(2) Kev Kho Mob: Rau PKU cov neeg mob tshwm sim los ntawm BH4 tsis muaj peev xwm, nws tuaj yeem siv ua lwm txoj kev kho. Los ntawm kev txhawb nqa, kev ua si ntawm PAH tuaj yeem rov qab los yog ib nrab ntawm cov ntshav phenylalanine ntau qib thiab txhim kho cov neeg mob chaw kho mob.
Lwm qhov BH4 qhov tsis txaus ntseeg
Ntxiv rau PKU, tseem muaj lwm yam kev teeb meem caj ces tshwm sim los ntawm BH4 tsis muaj peev xwm tshwm sim los ntawm Hyperthyroidism thiab Dopa teb DYSTONY (DRD). Cov neeg mob nrog cov kab mob no tseem tuaj yeem txo cov tsos mob thiab txhim kho lawv lub neej zoo los ntawm kev kho.
Pharmacological cuam tshuam thiab cov ntawv thov kev kho mob
Nws muaj ntau cov khoom siv hluav taws xob thiab cov txiaj ntsig zoo hauv cov ntawv thov kho mob.
Txhim kho enzyme
Raws li Cofactor ntawm ntau cov enzymes, nws tuaj yeem txhim kho cov haujlwm ntawm cov enzymes no, yog li nrawm qhov nqi ntawm kev sib txuam ntawm cov metabolites.
Txhim kho cov mob hauv tsev kho mob
Los ntawm kev txhawb nqaSapropterin hydrochloride, cov mob kho mob ntawm BH4 Cov Txwv tsis raug cai tuaj yeem txhim kho, xws li txo cov ntshav phenylalanine ntau thiab norepinephrine qib, thiab txhim kho cov haujlwm neurological.
Kev Nyab Xeeb thiab Ua Siab
Hauv kev sim tshuaj mob, nws tau pom muaj kev nyab xeeb thiab thev taus, thiab feem ntau muaj peev xwm tiv taus thiab tau txais txiaj ntsig los ntawm kev kho cov tshuaj no. Txawm li cas los xij, nws tseem tsim nyog los saib xyuas tus neeg mob lub siab thiab raum ua haujlwm raws li cov ntshav phenylalanine qib thaum siv.
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SapRopterin Hydrochloride CAS 69056-3-8-8Tsim los ntawm Bloom Tech XI'an City Huyi GMP Lub Tsev Haujlwm Saib Xyuas Kev Lag Luam.
Nco ntsoov: Ua tiav Chem - Tech yog tus tswv cuab tam sim ntawm peb, Bloom Tech (Txij xyoo 2008)
Raws li cov kauj ruam saum toj no, cov hauv qab no yog qhov tseem ceeb ntawm cov khoom noj:
1. Oxidation cov tshuaj tiv thaiv ntawm hydroxybenyyl cawv:
4-hydroxybenylenencoyl cawv → oxidation + c7h6o2
2. Sulfation cov kev tawm tsam ntawm Paba:
Kaba + So3 → Sulforation + C8H15No2
3. Txo cov tshuaj tiv thaiv ntawm Pabs:
C8h15no {2 + h2 → Txo + Pasas
4. Cyclization cov tshuaj tiv thaiv ntawm Pabas:
Pabas → Cyclization + C15h10o4
5. Hydrogenation cov tshuaj tiv thaiv ntawm DHF:
C15h10o 4 + h 50 → hydrogenation + c4h8o
6. Txo cov tshuaj tiv thaiv ntawm THF:
C4h8o + H2 → Txo + 9 h15n5o3
7. Hydrochlorination ntawm Bh4:
9H15n5o3 + hc → c9hh17Cl2n5o3
Los ntawm cov kauj ruam no, nws tuaj yeem ua tau zoo synthesized, thiab txhua kauj ruam cov tshuaj tiv thaiv los ntawm cov txheej txheem synthesis thiab cov tshuaj tiv thaiv zoo thiab kev ua kom dawb huv ntawm cov khoom.
Txoj kev rau synthesizing cov khoom tuaj yeem traced rov qab mus rau cov tshuaj synthesis ntawm tetrahydroid acid (thf) thiab nws cov derivfatolic acid (thf) thiab nws cov derivfatic.
Thawj kauj ruam yog los ua ke 4-hydroxybenzaldehyde. Nov yog cov khoom siv tseem ceeb uas tuaj yeem tau txais los ntawm cov txheej txheem tshuaj tiv thaiv hauv qab no:
Cov tshuaj tua kab mob oxidation: 4-hydroxybenyyl cawv tuaj yeem oxidized kom tau 4-hydroxybenzaldehyde.
4-hydroxybenyyl cawv → oxidation + 4- hydroxybenzaldehyde
Cov kauj ruam tom ntej koom nrog cov synthesis ntawm tetrathoredolle. TetraYDR hauv paus yog nws cov qauv kev coj ua, thiab nws cov synthesis feem ntau suav nrog cov kauj ruam hauv qab no:
Sulfation kev tawm tsam ntawm Paba:
para aminobenzoic acid (kaba) undergoes sulfation cov tshuaj tiv thaiv kom tau para aminobenzoyl sulfonic acid (pabs), pars {{} aminobenzoyl sulfate).
Kaba + So3 → Sulfonation + PABs
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Txo:
Ua cov tshuaj tiv thaiv kev txo qis ntawm cov phabs kom tsim p- o} aminobenzhlidoropane sulfenzoyl aminoprane sulfate).
Pabs + h2 → Txo + Pasab
02
Kev Cuam Tshuam Cyclization:
Pivas undergoes cyclization cov kev coj ua kom tsim cov acihydrofolic acid (DHF).
Pabas → Cyclization + DHF
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Hydrogenation txawv:
DHF undergoes txuas ntxiv hydrogenation cov tshuaj tiv thaiv kom tau tetrathydrofolle (thf).
DHF + H2 → Hydrogenation + Thf
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Tom qab tau txais tetrahydrofoforate (Thf), nws yuav tsum tau txo qis rau heptahydrodroorroly (7,8-dhihydrobiopterin, bh4). Cov kauj ruam no feem ntau cuam tshuam cov hauv qab no:
Txo: TetraYDR hauv tsev (THF) undergoes txo cov tshuaj tiv thaiv los ua cov tshuaj hatchayrfofolle (BH4).
THF + H2 → Txo + Bh4
Kauj ruam kawg yog kom rov qab tua HeptahyDRropetrhaydrofrolate (Bh4) nrog hydrochloric acid los ua lub fydrochloride daim ntawv ntawmSapropterin hydrochloride.
Hydrochlorination: heptahydrotetrahydrofofo (bh4) reacts nrog hydrochloric acid los tsim cov khoom.
Bh 4+ hc → c9hh17Cl2n5o3
Cim npe nrov: Sapropterin Hydrochloride CAS 69056-38-8, Cov chaw tsim khoom, chaw tsim khoom, chaw tsim khoom, yuav khoom, nqe, loj, rau muag