Melanin cells absence - Professional Look
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Providing the color of the skin, hair and eye iris pigment melanine synthesized by specialized cells-and-coming melanotsitami other cells in the skin. But it pigmentiruyutsya not all cells that make up the complex multilayer structure of the skin. Scientists Massachusetskoy Hospital (d. Boston), working under the direction of Dr. Janice Brissette (Janice Brissette), partially rasshifrovali sharing arrangements between melanin skin layers Melanin cells absence.
In humans, melanine is contained in the cells of the skin and hair. At the same time, some mammals, including mice, he found exclusively in wool, leather while still nepigmentirovannoy. Melanotsity melanine distribute via cell membrane protrusions - dendrite, which interact with other types of cells that make up the epidermis (outer layer of skin) or hair follicle. However, the mechanism that defines kletki-nakopiteli melanin, thus far remained unknown and Melanin cells absence.
The authors suggested that a role in the mechanism belongs genes Foxn1, the lack of which is characteristic of the so-called "naked" mice, which strand of wool skin is so thin that they oblamyvayutsya, leaving animals already have defective skin cover virtually lysymi "Melanin cells absence" . In humans inactivate gena-analoga Foxn1 led to the development of such a syndromic.
Gilchrest, Barbara A. (Brookline, MA, US)
Melanin cells absence - Details
Melanin is a special pigment that gives the skin and hair its natural color. It makes the skin tanned, but also produces unwanted freckles, birthmarks and other colored marks on the body.Melanin cells absence - More Details
With humans melanin forms in special sea-star shaped cells called melanocytes in the basal layer of epidermis and then transforms into granules where pigments are combined with protein. Melanocytes inject melanin grains into the cells of the upper skin layer (cortical layer of the hair and the iris of the eye) and determine their color.Melanin cells absence - Doctors view
Doctors from time to time state that tea, coffee, chocolate and cacao are harmful for our health. However, it is no good to give up these products once and for all as they contain melanin that is essential for the human organism. Also prunes, bilberry or dark grapes are good protection against cancer, sunburns and influence of radiation.Scientists have created transgenic mice expressing distinct abnormal gene Foxn1 cells not normally contain melanine. Such animals usually Melanin cells absence stations acquired skin color. Study of animal skin samples showed that dendrity Melanin cells absence selectively provide melanin cells ekspressiruyuschie Foxn1. The analysis also showed human skin expression of human version Foxn1 in kletkah-retsipientah melanin. As a result of further experiments, researchers found that Foxn1 interacts with Melanin cells absence through protein Fgf2, which increases the cell activation when expression Foxn1.
The authors believe that, apart from attracting Melanin cells absence and transfer melanin, the mechanism Foxn1/Fgf2 performs other functions, and most likely, is not the only mechanism, the distribution manager pigment. In the near future they plan to take up the search for other genes involved in the formation of pigmentary phenotype organism. Over time, the results of this work should help in developing methods for the prevention and treatment of hair posedeniya diseases such as Vitiligo (Autoimmune disease, which occurs in certain areas of the skin discoloration) and melanomas (malignant tumor, emerging from Melanin cells absence).
By albinizmu true cluster, based on a hereditary metabolic defect in melanin, resulting in a reduction in the number of pigment, or lack of pigment in the skin, mucous shell, hair, eyes (13). Functional violations that accompany these diseases, not only due to inability to perform its protective functions of melanin, but a variety of other changes inherent in patients with albinos.
The normal process includes turning Melanin cells absence tirozina amino acids, with the participation of melanine tirozinazy enzyme. When albinizme happening intracellular metabolic block this path, and at various levels.
Gordon, Philip R. (Philadelphia, PA, US)
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Melanin cells absence - Good Hair
Having a good hair day doesn’t just happen by chance. It happens when taking care of your hair becomes just as important as taking care of your skin. But what do you do when your hair has been damaged by every day styling, and chemical processing, dyeing or perming? Is there any solution for fly-away hair, dullness and faded color?Melanin cells absence - Science view
The color of hair is determined by the amount of melanin in the cortical layer. Dark hair has a lot of pigment while fair hair has fewer granules that are of smaller size. Diffusive or non-granulated melanin makes the hair red. Hair, like any other part of the body, requires essentials nutrients for growth and survival.
Melanin cells absence - Good Skin
When summer looms, many people start considering the best way to get that sun-bronzed glow — turning to self-tanners, tanning booths, a stretch in the sun, or a combination of these.Melanin cells absence - Science view
But before you go into a tanning booth — spend a few minutes finding out about your skin and sun exposure. These facts can help you get the look you want without stressing your skin.Melanin cells absence - More Details
Melanin is the body's way of protecting skin from burning. Darker-skinned people tan more deeply than lighter-skinned people because their melanocytes produce more melanin. But just because a person doesn't burn does not mean that he or she is also protected against skin cancer and other problems.Melanin cells absence - See more
Think about what you've read so far. Does it reinforce what you already know about Melanin cells absence? Or was there something completely new? What about the remaining paragraphs about Melanin cells absence?Melanin cells absence - People view
Scientists have created transgenic mice expressing distinct abnormal gene Foxn1 cells not normally contain melanine. Such animals usually bespigmentnye stations acquired skin color. Study of animal skin samples showed that dendrity melanotsitov selectively provide melanin cells ekspressiruyuschie Foxn1. The analysis also showed human skin expression of human version Foxn1 in kletkah-retsipientah melanin. As a result of further experiments, researchers found that Foxn1 interacts with Melanin cells absence through protein Fgf2, which increases the cell activation when expression Foxn1.The authors believe that, apart from attracting Melanin cells absence and transfer melanin, the mechanism Foxn1/Fgf2 performs other functions, and most likely, is not the only mechanism, the distribution manager pigment. In the near future they plan to take up the search for other genes involved in the formation of pigmentary phenotype organism. Over time, the results of this work should help in developing methods for the prevention and treatment of hair posedeniya diseases such as Vitiligo (Autoimmune disease, which occurs in certain areas of the skin discoloration) and melanomas (malignant tumor, emerging from melanotsitov).
By albinizmu true cluster, based on a hereditary metabolic defect in melanin, resulting in a reduction in the number of pigment, or lack of pigment in the skin, mucous shell, hair, eyes (13). Functional violations that accompany these diseases, not only due to inability to perform its protective functions of melanin, but a variety of other changes inherent in patients with albinos.
The normal process includes turning Melanin cells absence tirozina amino acids, with the participation of melanine tirozinazy enzyme. When albinizme happening intracellular metabolic block this path, and at various levels. (114)
"Dilution" pigment in glazo-kozhnom albinizme happening because of the inadequate number melanosom melanizatsii normal. When tirozinazo-negativnom type glazo-kozhnogo albinizma this is due to the total absence tirozinazy, while other forms albinizma this enzyme is present in the body, but does not operate with sufficient effectiveness Melanin cells absence.
In melanine protects the skin surface vessels and the nucleus of cells malpigievyh solar radiation, and he acts as absorbent, which is under the action of light provides free electrons. The function of melanin in the eye is more extensive and complex and still not completely understood. But knowing that melanosomy parties are specific antioxidant defense system pigmentary cells epithelium rear legs eyes, as expressed possess antioxidant properties: they effectively linked prooksidantnye ions, in particular, the iron ions, and no less effectively interact with the active forms of oxygen. Lack of melanin from albinos leads perhaps as compensation, to raise almost twice the content of alpha pigmentary epithelium. In addition, melanine, which is part of the melanosom, absorbs light very efficiently, especially in the area of the ultraviolet spectrum. And because the exterior segments fotoretseptornyh cells surrounded sprouts pigmentary epithelium cells containing melanosomy, it is effectively absorbing scattered light inside a glass eye, these sprouts by playing the role of shielding gaskets, allowing the ability to raise our eyes. Consequently, the absence of blacks in melanosom pigmentary epithelium in albinizme fotofobii and leads to increased risk of light damage to the retina and pigmentary epithelium. Thus, melanosomy as makulyarny pigment, and perform optical and biochemical (antioxidant) functions in the retina and protection systems against the risk of pigmentary epithelium Melanin cells absence.
According to the Silver Melanin cells absence and Webster Melanin cells absence melanine plays a significant role in the formation and development of the nervous system, which indirectly confirmed the presence of persons with albinos in improper decussation hiazme visual nerve, malformed makulyarnoy area and the frequent combination albinizma with deaf. GIPOPIGMENTATSII CONDITION AND THEIR PATHOGENESIS. Albinism is a group of genetically deterministic violations melanin-pigmentnoy system. It also occurs in the animal world, and is the result of congenital metabolic irregularities. His main clinical characteristics: depigmentatsiya hair, eyes (glazo-kozhny albino), or just the eye (ocular albino), in combination with fovealnoy gipoplaziey, nistagmom, Melanin cells absence, reduced the severity of view and a violation stereopsisa.
Melanin cells absence - More Details
Think about what you've read so far. Does it reinforce what you already know about Melanin cells absence? Or was there something completely new? What about the remaining paragraphs about Melanin cells absence?Melanin cells absence - Science view
Virtually all forms of inherited glazo-kozhnogo albinizma Melanin cells absence type. Thus, both parents sick child can only be carriers of the state, but without clinically look healthy. The exception to this rule is a form of autosomno-dominantnaya glazo-kozhnogo albinizma very rare breach, which was described only in the two cases independently investigated. Eye albino has only two options inheritance: H-svyazanny and autosomno-retsessivny.There are also genetic evidence of heterogeneity albinizma. Some reported that the registered cases, when the couple with a child rozhdalsya albinos, the body which normally produtsiroval pigment. It has been shown that in those families in which the parents have suffered various forms albinizma (tirozinazo-pozitivnym and tirozinazo-negativnym), the two violations determiniruyutsya allelyami different. Part albinoticheskih states accounted for by gene mutations, determiniruyuschego education tirozinazy (38, 61, 103, 110).
It has been proved that the gene encoding active tirozinazy, responsible for the wrong decussation visual nerve fibers in Melanin cells absence.
Circulation albinizma worldwide was calculated Witkop and co. (110) in 1974, and evaluated as 1:17000. In examining the population glazo-kozhnogo albinizma USA found that tirozinazo-pozitivnaya form is the most common. This type albinizma also prevalent among Africans, Asians and Indians, and in England and tirozinazo-pozitivnaya Melanin cells absence forms occur equally, and cases H-svyazannogo eye albinizma occur with a frequency of 1:50000. But with the variety albinizma autosomno-retsessivnym type of inheritance has dominated. The authors recall that in the UK are about 4000 people suffering albinos. Unfortunately, in Russia proliferation albinizma frequency in the population studied, and not enough accurate data to impossible.
At the same time, though fairly rare albino pathology, in certain racial groups, there is an unusually high percentage of cases albinizma associated with a significant imbridingom. For example, Melanin cells absence syndrome is most frequent among Puerto Ricans.
The normal process includes turning Melanin cells absence tirozina amino acids, with the participation of melanine tirozinazy enzyme. When albinizme happening intracellular metabolic block this path (below provides a biochemical diagram illustrating education pigments) (Figure 1) - tirozinaza dopahromoksireduktaza and play specific roles in the pathogenesis albinizma. Tirozinaza involved in the early stages of education pigment, and is involved in education dopahromoksireduktaza cherno-korichnevogo pigment. While cysteine provides education krasno-zheltogo pigment. Melanin cells absence process occurs within melanosom.
"Dilution" pigment in glazo-kozhnom albinizme happening because of inadequate normal number melanosom melanizatsii (110115). When tirozinazo-negativnom type glazo-kozhnogo albinizma this is due to the total absence tirozinazy, while other forms of this enzyme is present in the body, but does not operate with sufficient efficiency (88). According to the Witkop, has found no evidence of melaninoobrazovaniya inhibitors, and the level and variety of incentives tirozina hormones were within the norm (112). At the same time, ocular albino is the result of a reduced number of eye Melanin cells absence.
Identification of options albinizma based on the genetic, biochemical and histological criteria. But it is not always possible otdifferentsirovat albinizma from one type to another in the face of the clinic. Biohimicheski can be allotted 2 most common type glazo-kozhnogo albinizma (using test lukovitse hair). The first of these is described Kugelman and Wan-Scott in 1961 g (76). In conducting this test svezhevydernutaya inkubiruetsya hair bulbs in a buffer solution tirozina over 24 hours, and then studied. During the incubation hair bulbs some albinos do not generate a pigment (that is demonstrated by the complete absence of the enzyme tirozinazy), while in other albinos onion growing concentration of melanin found. These forms are classified as Melanin cells absence albino and tirozinazo-pozitivny albino respectively, on the latest figures (104), tirozinazo-negativny albino, in turn, is divided into type 1-A, in which the enzyme activity completely missing, and type 1-B , in which the enzyme activity decreased significantly (so-called "yellow" type). This biochemical analysis feasible only in patients older than 5 years. In 1976, a test has been upgraded King and Witkop (73), it was included to quantify the activity of a single hair tirozinazy lukovitse. Van Dorp and sponsors in 1984, (105) significantly increased both biochemical tests and assumed that would be more correct tirozinazo-pozitivny glazo-kozhny albino depending on the category of Melanin cells absence availability Melanin cells absence.
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