Umsebenzi peroxisome. Zellweger syndrome

Namhlanje siyakumema ukuba ucinge umsebenzi peroxisome. Yintoni ixabiso babe endlwaneni? nathi sinikele ingqalelo oko ububonile sifo ngoncedo le organelle.

Sele kwintshayelelo, kucacile ukuba peroxisomes - it organelles. Yeseli, bona, kuba yinxalenye umzobi emangalisayo ephucukileyo zonke esingqongileyo. Kwanomntu ubunjwe kwiiseli, apho kukho inani elikhulu kakhulu. Kwaye iseli liyilwe iinxalenye kakhulu, kodwa bavumela ukuba baphile ubomi ozimeleyo, oko kukuthi, ukuhlanganisa nokudala izakhiwo ezintsonkothileyo ayikho imfuneko. Umzekelo woku izidalwa zilula single-ezineseli, apho weva kwizifundo ibhayoloji esikolweni.

Phambi kokuba uluhlu uze uchaze umsebenzi peroxisome, kufuneka siqonde ukuba yintoni na kunye nokuba zisebenza njani.

peroxisomes

Ezi organelles kuvela vacuoles abancinane, ubungakanani yabo ngezantsi 1.5 micron. Peroxisome engqongwe inwebu obhityileyo, kwaye esahlula microgranules ngaphakathi, ukususela bume lwangaphakathi yonke iseli. Yintoni esinokuyifumana ngaphakathi organelle? Kukho undoqo, ikwabizwa ngokuba nucleoid. Kubalulekile ukuqonda ukuba le nxalenye peroxisomes akukho ngokunxulumene isakhiwo yenyukliya okanye iintsholongwane nucleoid. Musa ukubhidanisa la magama. yaye ngokufuthi kangangokuba izakhiwo bakufumanisayo ezilinganisa izakhiwo ziikristale zone nucleoid. Bona akhiwa fibrils kunye neetyhubhu. Undoqo yale organelle owahluliweyo peroxisomes, uratoksidoza ngaphakathi kwayo. Le yenye enzymes. Ngoku sicebisa ukuba siqwalasele umsebenzi peroxisome kwiseli. Kodwa phambi koko, sihlolisise isakhiwo.

isakhiwo

Ngoku ke ndiya kukuxelela into peroxisomes, isakhiwo kunye nemisebenzi (jonga itheyibhile) nazo ziya kuqwalaselwa kuthi. Okokuqala sinikela uncedo olubonakalayo ngokohlobo itafile. Emva koko, bhala iinkcukacha isakhiwo kunye nomsebenzi peroxisomes. Le theyibhile anokwenziwa sithi, kodwa kwanele ukuba uqaphele ukuba isizalo peroxisome kwaye iquka a nucleotide.

Matrix 15 iqulethe a enzymes peroxisome. Makhe sijonge ezinye. Iphawula eyona ibaluleke yabo;

• peroxidase;
• catalase;
• oksidoza;
• uratroksidaza.

Kwaye yintoni umsebenzi ngoko haya nucleotide? indima yayo yokuba ijolise ngamafuphi ezo enzyme efanayo. Makhe sijonge ukuba inkqubo yokwakha peraksisom. Baya ityatyambile evela EPS, ngoko singatsho ukuba indawo kuveliswe le organelle - a CSE. Enzyme ziveliswa:

• EPS;
• hyaloplasm.

Apha kubalulekile ukucacisa into yokuba inwebu, nangona zingcakacile, kodwa oko akuthethi ukungena nkqu ion kunye substrates ephantsi ubunzima eziphilayo. Ngoku ke siqwalasele umsebenzi peroxisomes. Okokuqala le organelle oko ke kubaluleke gqitha ukuba iseli.

imisebenzi

Imisebenzi Peroxisome zezi zilandelayo:

• igcwala kwezinto eziphilayo;
• okunamafutha igcwala acid;
• plasmalogens biosynthesis;
• biosynthesis isoprenoid kwizilwanyana;
• biosynthesis of cholesterol;
• NDR2 protein eyendawo;
• ummiselo inflammation lwenkqubo;
• uncedo kule nkqubo nokuphefumla kwi plant cells, njalo-njalo.

Olu luhlu elide kakhulu, kuba abanye Olusebenziso okwangoku ziqondwa ngokupheleleyo. Ngoku enze kancinci ukuthetha protein yokuqaphela. Kubalulekile ukwazi ukuba ezi organelles ziqulathe iindawo ezifana:

• DNA;
• ribosome.

Ngako oko, le njini protein kwenzeka ngumongo. Okulandelayo ke ukubaluleka bume yangaphakathi peroxisomes. njani undoqo yokungenisa? Ngokuthabatha inxaxheba C-terminal umqondiso. Ulandelelwano yale sokukhomba bufutshane kakhulu, eziquka 3 iintsalela acid acid.

Ixabiso kuviwo clinical

Siye sahlolisisa kwicandelo elidlulileyo, apho umsebenzi wabo wenziwa peroxisomes. Ngoku kancinci malunga lwezonyango organelle data kuyo. Ngoku sixoxa kancinci malunga nesifo ekuthiwa - Zellweger syndrome. Oku kunika umdla kakhulu sisifo esibi, oko kubangelwa kophulwa sokungenisa protini. Ngenxa yoko, peroxisomes engenanto, iseli kunye nenyama xa iyonke elibhaqiweyo data organelles ukusilela oyingozi. Abantu abaphethwe kwezi bazijongela ubunzima kunye nomsebenzi kwamabhunga ebalulekileyo apha ziquka:

• ingqondo;
• isibindi;
• nezintso.

Abantwana abazalelwe ngale syndrome awukwazi kuphila ixesha elide kwaye kunyanzeleke ukuba ushiye ihlabathi lethu emva kokuhlala elifutshane kakhulu. Isifo kuhambisana kuguquka le organelle. Kwakhona iintlobo sifo kunokwenzeka yaye ngaphantsi yingozi.