Ubururu bwa metilene n'ibura rya G6PD: impamvu ibyago ari ingenzi

Ubururu bwa metilene ntibugomba gukoreshwa ku bantu bafite ibura rya enzime ya glukoze-6-fosifate dehidurojenaze, rikunze kwitwa ibura rya G6PD mu magambo ahinnye. Amakuru agenewe abatanga PROVAYBLUE agaragaza ko impamvu ari ukubura amaraso guterwa no gusenyuka kw'uturemangingo dutukura tw'amaraso. Ibi bivuze ko uturemangingo dutukura tw'amaraso dushobora gusenyuka vuba kurusha uko umubiri udusimbuza. Iryo bura rishobora kandi kubuza umuti gukora uko biteganyijwe mu kuvura methemoglobinemiya.

Ibi bibazo birafitanye isano. Ubururu bwa metilene bukenera ubushobozi bwo gutanga electron imbere mu karemangingo gatukura k'amaraso. G6PD ifasha kubungabunga ubwo bushobozi, ari na bwo akaremangingo gakenera kugira ngo kirinde kwangizwa na okisidasiyo. Kuba igicuruzwa gifite ubuziranenge ntibikosora ibura rya enzime mu muntu ugihawe.

Icyo G6PD ikora mu karemangingo gatukura k'amaraso

G6PD ni enzime iri mu ruhererekane rw'ibikorwa rwa pentoze fosifate, inzira yo gutunganya glukoze. Kimwe mu biva muri urwo ruhererekane ni NADPH, molekile itanga electron mu bindi bikorwa bya shimi. Izo electron zituma ibikorwa byinshi byo kurinda akaremangingo bikomeza gukora.

Amabwiriza ya CPIC ku miti n'imiterere y'ingirabuzima fatizo za G6PD asobanura impamvu ibi ari ingenzi cyane mu turemangingo dutukura tw'amaraso: urwo ruhererekane rwa pentoze fosifate ni rwo soko rya NADPH utwo turemangingo dufite. G6PD n'indi enzime, 6-fosifogulukonate dehidurojenaze, bikora NADPH muri urwo ruhererekane. Kugabanuka kw'imikorere ya G6PD kugabanya ubushobozi bw'akaremangingo bwo guhangana n'igitutu cya okisidasiyo iyo kiyongereye.

Kimwe mu bikorwa byo kurinda akaremangingo bikoresha NADPH ni ukongera gutunganya glutatiyoni kugira ngo yongere gukoreshwa. Glutatiyoni redukitaze ikoresha NADPH mu gusubiza glutatiyoni mu miterere yakiriye electron, ibyo bikaba bifasha glutatiyoni perokisidaze gukuraho perokiside. Ibisobanuro bya PharmGKB ku ruhererekane rw'ibikorwa mu turemangingo dutukura tw'amaraso bigaragaza isano y'ibi bikorwa. Iyo ubushobozi bwo gutanga electron budahagije, ibintu bitera okisidasiyo bishobora kwangiza hemoglobine n'akaremangingo iyirimo.

Bityo, ibura rya G6PD rireba ubushobozi bwo guhangana n'ikintu gishobora kwangiza akaremangingo. Ntibivuze ko buri muntu urifite ahora afite ikibazo cyo kubura amaraso cyangwa yumva arwaye buri munsi.

Impamvu ubururu bwa metilene bushingira kuri ubwo bushobozi

Ubusanzwe hemoglobine irimo ubutare buri mu miterere ya ferusi, Fe²⁺. Okisidasiyo ibuhindura mu miterere ya feriki, Fe³⁺, bigatuma haboneka methemoglobine. Ubwo butare bwahindutse ntibushobora gufata ogisijeni uko bisanzwe. Iyo methemoglobine ibaye nyinshi cyane, kugeza ogisijeni mu mubiri birabangamirwa.

Ubururu bwa metilene bushobora gufasha gusubiza iyo miterere uko yari imeze, ariko hari intambwe yo hagati y'ingenzi. Imbere mu karemangingo, kwakira electron bishingiye kuri NADPH bihindura ubururu bwa metilene bukaba ubururu bwa leukometilene. Ubururu bwa leukometilene na bwo butanga electron kuri methemoglobine, bukayisubiza ku miterere ya hemoglobine ikora neza. Uru ruhererekane rusobanurwa mu bisobanuro by'uko umuti ukora biri ku nyandiko yawo.

Hari kandi urundi ruhererekane rusanzwe rwo kubungabunga imikorere: sitokorome b5 redukitaze ishingiye kuri NADH ikora igice kinini cyo guha electron methemoglobine iba ihari mu buryo busanzwe. NADH na NADPH ni molekile zitandukanye. Ibura rya G6PD ntirihagarika gusa inzira zose zo kubungabunga hemoglobine. Ikibazo kirushaho kuba ingenzi iyo guhura n'ikintu gitera okisidasiyo byongereye ibikenewe, maze ubururu bwa metilene bugatangwa. Inyandiko yacu ku bururu bwa metilene mu kuvura methemoglobinemiya isobanura uko bukoreshwa mu buvuzi.

Ingaruka ebyiri za NADPH idahagije

Igishushanyo 1. Igishushanyo cyoroheje cy'isano y'ibikorwa bishingirana. Imyambi ihuza udusanduku tw'uruhererekane igaragaza inkunga igikorwa gihabwa; ntabwo ari urutonde rwuzuye rw'ibikorwa bya shimi. Mu ishami ry'umuti, ubururu bwa leukometilene butanga electron zikenewe mu guhindura Fe³⁺ ikaba Fe²⁺. Ubururu bwa metilene ubwabwo bushobora kongera igitutu cya okisidasiyo. Iki gishushanyo gisobanura igitekerezo gihuza amakuru agenewe abatanga umuti n'uruhererekane rwa PharmGKB ruri mu ihuza.

Inama za EHA na EuroBloodNet zigaragaza ko ibura rya G6PD rishobora kugabanya imikorere y'umuti no guteza ibyangiritse:

1. Ubuvuzi bushobora kunanirwa. NADPH idahagije ishobora kugabanya ihinduka ry'ubururu bwa metilene mu miterere yabwo itanga electron, methemoglobine ntikosorwe bihagije.
2. Kwangizwa na okisidasiyo bishobora kwiyongera. Ubururu bwa metilene ubwabwo ni ikintu gitera okisidasiyo. Mu karemangingo gafite intege nke, bushobora kugira uruhare mu gusenyuka kw'uturemangingo dutukura tw'amaraso kandi bugatuma methemoglobinemiya irushaho gukomera.

Izi ngaruka zishobora kubaho icyarimwe: uturemangingo dutukura tw'amaraso tutangiritse tukaba duke, kandi n'utusigaye tukagira ubushobozi buke bwo gutwara ogisijeni. Ni yo mpamvu kutabona igisubizo gihagije ku muti atari impamvu yo gukomeza kongera ubururu bwa metilene ku giti cyawe.

Impamvu kumva umeze neza bitagaragaza ko nta byago bihari

Dukurikije MedlinePlus Genetics, abantu benshi bafite ibura rya G6PD nta bimenyetso bagira kandi ntibazi ko barifite. Indwara ziterwa na mikorobe, imiti imwe n'imwe n'ibindi bitera okisidasiyo bishobora gutuma izo ntege nke zigaragara. Nubwo iyi miterere ifitanye isano n'ihererekanywa ry'irage rishingiye kuri koromozome X kandi ikaba ikunze kugaragara mu bagabo, abagore na bo bashobora kugira ibura rifite akamaro mu buvuzi.

Ikintu cyagaragaye mu igerageza gifasha kwerekana itandukaniro hagati y'imiterere isanzwe n'igihe akaremangingo kari mu gitutu. Mu bushakashatsi bwo mu 1996 bwa Bozzi, Parisi na Strom, uturemangingo dutukura tw'amaraso y'abantu twari tumaze gukusanywa kandi dufite ibura rikomeye rya G6PD twari dufite urugero rwa glutatiyoni yakiriye electron rwegereye urwo mu turemangingo dusanzwe. Ariko nyuma yo kudushyira mu igerageza ryakoresheje perokiside, uturemangingo dufite iryo bura twatakaje glutatiyoni ku buryo bugaragara kandi ntitwashoboye kwisubiza neza ugereranyije n'uturemangingo dusanzwe. Aya yari amagerageza yo muri laboratwari ku turemangingo tw'amaraso, si igerageza ryagaragazaga ingano y'ubururu bwa metilene itekanye. Ibyabonetse bisobanura impamvu imiterere ibanza isa n'isanzwe idashobora byanze bikunze kwerekana ubushobozi bwo guhangana n'ikintu umuntu azahura na cyo.

Icyo wakora ukoresheje aya makuru

Niba warasuzumwe ukemezwa ko ufite ibura rya G6PD, bibwire umuganga cyangwa umuhanga mu by'imiti mbere y'uko hateganywa gukoresha ubururu bwa metilene. Amabwiriza ya CPIC ashyira ubururu bwa metilene mu miti ifite ibyago byinshi. Kuba umucuruzi abuvuga nk'ubukoreshwa mu ngano nto cyangwa kuba warigeze kubukoresha ntihagire ikibazo bigutera, ntabwo ari uruhushya rwo kwirengagiza ko butagomba gukoreshwa.

Niba utazi neza uko G6PD yawe ihagaze, zana ibisubizo byose bya G6PD wigeze kubona n'amakuru ajyanye n'iki kibazo mu muryango wawe igihe ubiganiraho n'abaganga. Gusenyuka kw'uturemangingo dutukura tw'amaraso cyangwa guterwa amaraso biherutse kuba bishobora kugira ingaruka ku gusobanura ibisubizo by'igipimo cya enzime. Inyandiko iyobora ku gupima G6PD muri laboratwari isobanura icyo gipimo n'aho ubushobozi bwacyo bugarukira; igipimo gisanzwe cyo kubara uturemangingo tw'amaraso cyonyine ntigisubiza icyo kibazo. Uko G6PD ihagaze na byo ni igice kimwe gusa cy'isuzuma ryagutse ry'umutekano w'ubururu bwa metilene.

Nyuma yo guhura n'ubururu bwa metilene, intege nke nshya zikomeye, guhumeka bigoranye, umuhondo ku ruhu cyangwa mu maso, inkari zijimye cyangwa umutima utera vuba bisaba isuzuma ryihuse kwa muganga. Guhumeka bigoranye cyane, kubabara mu gituza, kugwa hasi cyangwa kujijwa bisaba ubuvuzi bw'ubutabazi. Zana igikoresho kirimo igicuruzwa cyangwa amakuru yacyo, harimo ubwinshi bw'ikinyabutabire muri cyo, ingano wakoresheje n'igihe wagihuriye na cyo. Ntukoreshe ibara ry'inkari ryonyine kugira ngo wemeze niba uturemangingo dutukura tw'amaraso turi gusenyuka.

Iyo methemoglobinemiya ibaye ku muntu ufite ibura rya G6PD, guhitamo ubuvuzi ni inshingano y'itsinda ry'ubuvuzi bw'ubutabazi. Inama z'inzobere zivuga ku bundi buryo bushobora gukoreshwa, harimo aside asikorubike ndetse, mu bihe bikomeye, uburyo bwo gusimbuza amaraso y'umurwayi andi maraso. Ibi ntabwo ari uburyo bwo kwivurira mu rugo umuntu ashobora gusimburanya uko ashaka: aside asikorubike ikora buhoro kurushaho, kandi uko ogisijeni igera mu mubiri w'umurwayi, uko uturemangingo dutukura tw'amaraso dusenyuka n'uko umurwayi ameze ni byo bigena ubufasha bukenewe.