Author/Editor		Kansky, Aleksej
Title		Novosti v procesu keratinizacije
Translated title		Noveltyes in process of keratinization
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 68, št. Suppl 2
Publication year		1999
Volume		str. II-3-6
Language		slo
Abstract		Background. Differentiation of human epidermal cells (keratinocytes) is a very complex process which includes 1. keratins, 2. desmosomes, 3. cornified cell envelop, 4. epidermal lipids as well as 5. the signaling system. Conclusions. Keratin are the main component of the cytoskeleton of keratinocyte, their structure is however altering from the basal up to cornified laver. A number of mutations affecting keratins K1, K2e, K5, K6a, K9, K10 and K14 have been recognized as causes of various hereditary disorders of keratinization. Desmosomes are in a broader sense coinvolved in the keratinization process. A normal structure and a normal process of breakdown are essential in the formation tge cornified layer. Certain disorders due to deficient desmosomes are included into hereditary disorders of keratinization. Cornified cell envelopes (CE) are essential for an appropriate function of the cornified layer. Mutations of loricin, one of the components of CE are responsible for the development of Vohwinkel's syndrome. Epidermal lipids are involved in the process of keratinization and are also essential for the barrier function. SImilar to keratins the lipida are also changing from polar to non-polar in the outer-most cornified layer. Signaling system is responsible for reception of signals at the cell-surface for the transmission through cytoplasm to the nucleus.
Summary		Izhodišča. Diferenciacija epidermalnih celic (keratinocitov) v širšem pomenu obsega več sistemskih procesov, ki vključujejo obnovo 1. keratinov in 2. dezmosomov, 3. nastanek beljakovinske membrane korneocitov ter 4. presnovo epidermalnih lipidov. Naštete procese uravnavajo 5. zapleteni signalni sistemi. Zaključki. Keratini, ki predstavljajo skelet epidermalnih celic spreminjajo svojo strukturo ob bazalne do rožene plasti. Znanih je več mutacij genov za keratine K1, K2e, K5, K6a, K9, K10, K14, K16 ter K17, ki povzročajo nastanek nekaterih dednih bolezni. Dezmosomi, ki jih sicer sestavljajo druge beljakovine, so tako tesno povezani s procesom keratinizacije, da jih je tudi potrebno obravavati v tem sklopu. Nekatere bolezni, ki nastanejo zaradi okvarjenih dezmosomov, vsaj v kliničnem smislu prištevamo med bolezni keratinizacije. Membrane korneocitov so pomembne za tako imenovano pregradno funkcijo rožene plasti. Znano je tudi, da mutacije nekaterih sestavin povzročajo nekatere dedne bolesni. Epidermalni lipidi sodelujejo tako pri procesu keratinizacije kot tudi pri pregradnem delovanju rožene plasti. Podobno kot velja za keratine, se tudi sestava lipidov spreminja od bazalne do rožene plasti. Signalni sistem. Receptorji na površini celic sprejemajo razne signale iz okolice ter jih posredujejo v citoplazmo, od koder se nato prevajajo v jedra celic.
Descriptors		KERATINOCYTES CELL DIFFERENTIATION KERATIN DESMOSOMES SIGNAL TRANSDUCTION EPIDERMIS TRANSCRIPTION FACTORS EPIDERMAL GROWTH FACTOR-UROGASTRONE