Peroxisome muaj nuj nqi. Zellweger syndrome

Niaj hnub no peb caw koj mus xav txog peroxisome muaj nuj nqi. Yuav ua li cas tus nqi lawv muaj nyob rau hauv lub tawb? peb kuj tau them nyiaj mloog mus rau dab tsi tus kab mob no yuav raug kuaj nrog rau cov kev pab los ntawm no organelle.

Twb tau nyob rau hauv phau, nws yog tseeb hais tias cov peroxisomes - nws organelles. Lub cell, nyob rau hauv lem, yog ib feem ntawm ib tug heev kawg sophisticated designer ntawm tag nrho cov uas nyob ib ncig peb. Txawm tus txiv neej yog tsim los ntawm cov hlwb, uas muaj ib tug heev loj tus naj npawb. Thiab lub cell yog ua los ntawm txawm me me, tiam sis lawv cia nws mus ua ib tug neeg sab nraud lub neej, uas yog, sib sau ua ke thiab tsim txoj lug yog tsis tsim nyog. Ib qho piv txwv ntawm no yog qhov nyuaj ib leeg-celled creatures, uas tej zaum koj hnov hauv ib zaj lus qhia, tom tsev kawm ntawv.

Ua ntej peb yuav sau thiab piav qhia txog cov peroxisome muaj nuj nqi, peb yuav tsum to taub nws yog dab tsi thiab yuav ua li cas lawv ua hauj lwm.

peroxisomes

Cov organelles tshwm sim raws li me me vacuoles, lawv loj hauv qab no yog 1.5 micron. Peroxisome surrounded los ntawm ib tug nyias daim nyias nyias, thiab nws separates lub microgranules sab hauv, los ntawm lub sab hauv ib puag ncig ntawm tag nrho cov cell. Yuav ua li cas peb yuav nrhiav tsis pub dhau lub organelle? Muaj cov tub ntxhais, nws tseem hu ua lub nucleoid. Nws yog ib qho tseem ceeb kom nkag siab txog qhov tseeb hais tias qhov no yog ib feem ntawm peroxisomes muaj tsis muaj kev sib raug zoo mus rau lub nuclear qauv los nucleoid kab mob. Tsis txhob xav yuam kev rau cov tswv yim. thiab feem ntau nws yog li ntawd lub kuaj lug mimicking muaju nucleoid tsam. Lawv yog ua los ntawm fibrils thiab hlab. Cov tub ntxhais ntawm no organelle sib cais los ntawm lub peroxisomes, uratoksidoza muaj nyob hauv nws. Qhov no yog ib tug ntawm cov enzymes. Tam sim no peb qhia mus xav txog qhov peroxisome muaj nuj nqi nyob rau hauv lub cell. Tab sis ua ntej ntawd, ib tug los ze zog saib cov qauv.

qauv

Tam sim no peb yuav qhia rau dab tsi peroxisomes, qauv thiab kev khiav dej num (saib cov lus) yuav kuj pom tau tias los ntawm peb. Yuav pib nrog peb muab ib tug yeeb yam pab nyob rau hauv daim ntawv ntawm ib lub rooj. Tom ntej no, sau tawm rau hauv kom meej cov qauv thiab cov kev ua ntawm peroxisomes. Lub rooj yuav tsum tsim los ntawm peb, tab sis suffice nws yuav tau nco ntsoov hais tias tus peroxisome matrix thiab muaj ib tug nucleotide.

Matrix 15 muaj ib tug peroxisome enzymes. Cia saib ib co. Qhov tseem ceeb qhov tseem ceeb ntawm lawv yog:

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

Thiab yog dab tsi muaj nuj nqi ces ua ib nucleotide? Nws lub luag hauj lwm yog rau condense cov tib enzymes. Cia saib rau tus txheej txheem ntawm txoj kev peraksisom. Lawv pib tawg paj los ntawm cov EPS, li ntawd, peb yuav hais tias cov cheeb tsam ntawm zus tau tej cov no organelle - ib tug CSE. Enzymes yog ua nyob rau hauv:

• EPS;
• hyaloplasm.

Ntawm no nws yog ib qho tseem ceeb hais qhov tseeb hais tias daim nyias nyias, tab sis yog nyias, tab sis nws tsis txeem txawm lub ions thiab ib tug tsawg molecular yuag substrates. Tam sim no peb muaj peev xwm xav txog qhov muaj nuj nqi ntawm peroxisomes. Nyuam no organelle nws yog ib yam uas tseem ceeb heev rau lub cell.

zog

Peroxisome zog yog raws li nram no:

• oxidation ntawm cov organic tshuaj;
• fatty acid oxidation;
• biosynthesis plasmalogens;
• isoprenoid biosynthesis nyob rau hauv cov tsiaj;
• biosynthesis roj uas txhaws taus;
• NDR2 protein localization;
• kev cai ntawm muaj quag o;
• kev pab nyob rau hauv tus txheej txheem ntawm kev ua pa nyob rau hauv cog hlwb, thiab hais txog.

Qhov no daim ntawv teev yog ntev heev, vim hais tias ib co zog yog tsis tau tag nrho to taub. Tam sim no peb muaj ib tug me ntsis ntau tham txog protein synthesis. Nws yog ib qho tseem ceeb kom paub hais tias cov organelles muaj qhov chaw xws li:

• DNA;
• ribosome.

Raws li, qhov protein synthesis tshwm sim nyob rau hauv lub nucleus. Tom ntej no yog cov ntshuam ntawm lub sab hauv ib puag ncig ntawm peroxisomes. Yuav ua li cas puas cov tub ntxhais ntawm lub ntshuam? Los ntawm cov C-davhlau ya nyob twg lub teeb liab. Cov kab sab ntawm no targeting yog heev luv luv, muaj raws ntawm 3 amino acid residues.

Tus nqi nyob rau hauv lub soj ntsuam kev xeem

Peb tau pom tias nyob rau hauv lub yav dhau los seem, uas muaj nuj nqi yog ua los ntawm peroxisomes. Tam sim no ib tug me ntsis txog cov kev soj ntsuam cov ntaub ntawv lo lus uas peb organelle. Tam sim no peb tham ib tug me ntsis txog tus kab mob hu ua - Zellweger syndrome. Qhov no yog nej ib tug kab mob loj, nws yog tshwm sim los ntawm ib tug ua txhaum ntawm protein ntshuam. Thiaj li, peroxisomes npliag, lub cell thiab tus kab mob raws li ib tug tag nrho kuaj cov ntaub ntawv mob tsis ua hauj lwm organelles. Cov neeg uas raug kev txom nyem los ntawm cov mob muaj teeb meem nrog lub chaw ua hauj lwm ntawm ib co tseem ceeb lub cev no muaj xws li:

• lub hlwb;
• siab;
• ob lub raum.

Cov me nyuam uas yug los nrog rau qhov no syndrome yuav tsis nyob ib lub sij hawm ntev thiab yog yuam kom khiav tawm peb lub ntiaj teb tom qab ib tug heev luv luv nyob. Tus kab mob yog txuam nrog ib tug kev hloov ntawm lub organelle. Nws tseem yog tau thiab tsawg txaus ntshai ntaub ntawv ntawm tus kab mob.