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Hedgehog (v překladu „ježek“) je genová ro … Hedgehog (v překladu „ježek“) je genová rodina zahrnující u obratlovců tři geny, (Dhh), (Ihh) a (Shh). Geny této rodiny však mají i bezobratlí a nejčastěji se studují na octomilkách. Jejich produkty, signální proteiny, se účastní stejnojmenné signalizační kaskády hedgehog. Tyto bílkoviny obsahují navázaný cholesterol a mastnou kyselinu (jedná se tedy o lipoproteiny). Hedgehog proteiny se vážou na membránový receptor (spřažený s G proteinem). Když to udělají, Patched přestane inhibovat další transmembránový protein, Smoothened (rovněž GPCR), který následně signál převádí dovnitř buňky. Z výzkumů na octomilce je zřejmé, že se po aktivaci Patched přestane protein (Ci) rozkládat v proteazomech. Díky tomu může Ci vstoupit do jádra a aktivovat zde určité geny, které jsou pod jeho kontrolou. Signalizace pomocí hedgehog proteinů má napříč živočišnou říší řadu funkcí. U octomilky řídí vývoj křídel, u ploštěnek regeneraci a u savců vývoj mozku, trávicí soustavy a prstů.vců vývoj mozku, trávicí soustavy a prstů.
, Der Hedgehog-Signalweg ist ein Signaltrans … Der Hedgehog-Signalweg ist ein Signaltransduktionsweg, durch den Zellen auf äußere Signale reagieren können. Der Signalweg ist nach seinem Liganden Hedgehog (Hh) benannt, einem Signalprotein, das als lokales Morphogen eine wichtige Funktion bei der Embryonalentwicklung von Tieren einnimmt und in der Evolutionsgeschichte sehr früh auftrat. Eine Fehlfunktion dieses Signalwegs führt zu massiven Fehlbildungen im Laufe der Embryonalentwicklung und kann bei Erwachsenen Krebs verursachen. In Säugetieren sind zurzeit drei Hedgehog-Proteine bekannt (sonic hedgehog, indian hedgehog und desert hedgehog).hog, indian hedgehog und desert hedgehog).
, Hedgehog (en français : hérisson), est une … Hedgehog (en français : hérisson), est une protéine. Elle est présente chez les invertébrés (c'est une protéine de polarité segmentaire, impliquée dans le contrôle des patrons de développement embryonnaire). Il y a 3 gènes homologue du gène Hedgehog chez les vertébrés. La voie de signalisation de Hegdehog est complexe et fait intervenir plusieurs autres protéines. Patched et Wingless, notamment, sont aussi nécessaires à la segmentation et à la différenciation et permettent la régulation de l’expression de Hedgehog. Il y a plusieurs homologues du gène hedgehog chez les vertébrés, comme Sonic hedgehog, Indian Hedgehog et Desert Hedgehog, tous aussi importants pour le développement embryonnaire. Plusieurs maladies sont d’ailleurs associées à la mutation du gène. L’expression ectopique du gène est en effet associée chez les vertébrés à des tumeurs au niveau de la peau, des glandes mammaires et du système nerveux. De plus, des conséquences fatales ont été observées chez des fœtus humains dont la voie de hedgehog était déréglée. Par exemple, des défauts de la croissance du cerveau et de plusieurs autres organes. Même à l’état adulte, hedgehog reste important pour la réparation des tissus et le bon renouvellement des cellules.sus et le bon renouvellement des cellules.
, The Hedgehog signaling pathway is a signal … The Hedgehog signaling pathway is a signaling pathway that transmits information to embryonic cells required for proper cell differentiation. Different parts of the embryo have different concentrations of hedgehog signaling proteins. The pathway also has roles in the adult. Diseases associated with the malfunction of this pathway include cancer. The Hedgehog signaling pathway is one of the key regulators of animal development and is present in all bilaterians. The pathway takes its name from its polypeptide ligand, an intracellular signaling molecule called Hedgehog (Hh) found in fruit flies of the genus Drosophila; fruit fly larva lacking the Hh gene are said to resemble hedgehogs. Hh is one of Drosophila's segment polarity gene products, involved in establishing the basis of the fly body plan. Larvae without Hh are short and spiny, resembling the hedgehog animal. The molecule remains important during later stages of embryogenesis and metamorphosis. Mammals have three Hedgehog homologues, Desert (DHH), Indian (IHH), and Sonic (SHH), of which Sonic is the best studied. The pathway is equally important during vertebrate embryonic development and is therefore of interest in evolutionary developmental biology. In knockout mice lacking components of the pathway, the brain, skeleton, musculature, gastrointestinal tract and lungs fail to develop correctly. Recent studies point to the role of Hedgehog signaling in regulating adult stem cells involved in maintenance and regeneration of adult tissues. The pathway has also been implicated in the development of some cancers. Drugs that specifically target Hedgehog signaling to fight this disease are being actively developed by a number of pharmaceutical companies.d by a number of pharmaceutical companies.
, ヘッジホッグシグナル伝達経路（- でんたつけいろ、英：Hedgehog signal … ヘッジホッグシグナル伝達経路（- でんたつけいろ、英：Hedgehog signaling pathway）は、ヘッジホッグタンパク質を中心としたシグナル伝達経路である。発生している胚では頭側・尾側、右側・左側などの位置が決定されそれに基づいてそれぞれ異なった発生過程をたどり、 またいくつかの分節を形成しそれぞれが異なった形に発達する。胚が正常に発生できるようにおのおのの細胞は「ヘッジホッグシグナル伝達経路」に基づいてこのような正しい位置情報を獲得する。すなわち、胚では場所に応じて異なった濃度のヘッジホッグシグナルを構成するタンパク質が見られるのである。発生過程のみならず成体においてもこのシグナル伝達経路は働いている。 ここに異常が生じると、基底細胞癌のような疾患を発症する。 ヘッジホッグシグナル伝達経路は、動物の発生に関わる重要な経路の1つであり、ショウジョウバエからヒトにいたるまで保存されている。この経路の名前はショウジョウバエで見つかったヘッジホッグ（Hh）と呼ばれるペプチド分子に基づくが、この分子はショウジョウバエの胚の極性を決める遺伝子（セグメントポラリティー遺伝子）の1種である。ヘッジホッグは後期の胚発生や変態でも重要な役割を担っている。 哺乳類ではソニック・ヘッジホッグ、、の3種類のヘッジホッグホモログが知られているが、一番研究されているのは全身に発現が見られるソニック・ヘッジホッグである。ヘッジホッグシグナルは脊椎動物においても胚発生で重要な役割を担っており、例えば、この経路を構成する遺伝子を欠くノックアウトマウスでは、脳や骨、消化管、肺をうまく形成することが出来ないことが知られている。近年の研究によるとヘッジホッグシグナルは体性幹細胞の制御に関わっており、成人組織の維持や再生でも働いているとされる。加えて、腫瘍の進展にもこのシグナルが関係していることがわかってきており、製薬会社はヘッジホッグシグナルを標的とした抗腫瘍薬の開発に力を入れている。きており、製薬会社はヘッジホッグシグナルを標的とした抗腫瘍薬の開発に力を入れている。
, A via de sinalização Hedgehog dá às célula … A via de sinalização Hedgehog dá às células embrionárias informações que lhes permitem fazer o embrião desenvolver-se de maneira correta, de modo que as células desenvolvem-se diferentemente na cabeça ou na cauda, à direita ou esquerda, e em outras posições. Também formam segmentos que se desenvolvem em diferentes partes do corpo. Diferentes partes do embrião possuem diferentes concentrações das proteínas de sinalização hedgehog, o que serve como sinal para diferenciação celular. A via também desempenha um papel em adultos. Quando a via funciona mal, pode provocar doenças, como o carcinoma de células basais. A via de sinalização hedgehog é um dos reguladores chave do desenvolvimento animal, conservado desde as moscas até aos humanos. A via toma o nome do seu ligando proteico, uma molécula de sinalização intercelular chamada Hedgehog (Hh) encontrada em moscas-da-fruta do género Drosophila. Hh é um dos produtos génicos de polaridade de segmentos em Drosophila, envolvida no estabelecimento das bases do plano corporal. A molécula permanece importantes nos estágios posteriores da embriogénese e metamorfose. Os mamíferos possuem três homólogos de Hedgehog, dos quais Sonic hedgehog é o mais estudado. A via é igualmente importante durante o desenvolvimento embrionário em vertebrados. Em ratos knockout, em que existe falta de componentes da via, o cérebro, esqueleto, musculatura, tracto gastrointestinal e pulmões falham em desenvolver-se correctamente. Estudos recentes apontam para o papel da sinalização hedgehog na regulação das envolvidas na manutenção e regeneração dos tecidos biológicos de adultos. A via também tem sido implicada no desenvolvimentos de alguns cancros. Drogas que especificamente têm como alvo a sinalização hedgehog, para combater esta doença estão a ser activamente desenvolvidas por companhias farmacêuticas.esenvolvidas por companhias farmacêuticas.
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| rdfs:comment |
A via de sinalização Hedgehog dá às célula … A via de sinalização Hedgehog dá às células embrionárias informações que lhes permitem fazer o embrião desenvolver-se de maneira correta, de modo que as células desenvolvem-se diferentemente na cabeça ou na cauda, à direita ou esquerda, e em outras posições. Também formam segmentos que se desenvolvem em diferentes partes do corpo. Diferentes partes do embrião possuem diferentes concentrações das proteínas de sinalização hedgehog, o que serve como sinal para diferenciação celular. A via também desempenha um papel em adultos. Quando a via funciona mal, pode provocar doenças, como o carcinoma de células basais.enças, como o carcinoma de células basais.
, Der Hedgehog-Signalweg ist ein Signaltrans … Der Hedgehog-Signalweg ist ein Signaltransduktionsweg, durch den Zellen auf äußere Signale reagieren können. Der Signalweg ist nach seinem Liganden Hedgehog (Hh) benannt, einem Signalprotein, das als lokales Morphogen eine wichtige Funktion bei der Embryonalentwicklung von Tieren einnimmt und in der Evolutionsgeschichte sehr früh auftrat. Eine Fehlfunktion dieses Signalwegs führt zu massiven Fehlbildungen im Laufe der Embryonalentwicklung und kann bei Erwachsenen Krebs verursachen. In Säugetieren sind zurzeit drei Hedgehog-Proteine bekannt (sonic hedgehog, indian hedgehog und desert hedgehog).hog, indian hedgehog und desert hedgehog).
, The Hedgehog signaling pathway is a signal … The Hedgehog signaling pathway is a signaling pathway that transmits information to embryonic cells required for proper cell differentiation. Different parts of the embryo have different concentrations of hedgehog signaling proteins. The pathway also has roles in the adult. Diseases associated with the malfunction of this pathway include cancer.alfunction of this pathway include cancer.
, ヘッジホッグシグナル伝達経路（- でんたつけいろ、英：Hedgehog signal … ヘッジホッグシグナル伝達経路（- でんたつけいろ、英：Hedgehog signaling pathway）は、ヘッジホッグタンパク質を中心としたシグナル伝達経路である。発生している胚では頭側・尾側、右側・左側などの位置が決定されそれに基づいてそれぞれ異なった発生過程をたどり、 またいくつかの分節を形成しそれぞれが異なった形に発達する。胚が正常に発生できるようにおのおのの細胞は「ヘッジホッグシグナル伝達経路」に基づいてこのような正しい位置情報を獲得する。すなわち、胚では場所に応じて異なった濃度のヘッジホッグシグナルを構成するタンパク質が見られるのである。発生過程のみならず成体においてもこのシグナル伝達経路は働いている。 ここに異常が生じると、基底細胞癌のような疾患を発症する。 ヘッジホッグシグナル伝達経路は、動物の発生に関わる重要な経路の1つであり、ショウジョウバエからヒトにいたるまで保存されている。この経路の名前はショウジョウバエで見つかったヘッジホッグ（Hh）と呼ばれるペプチド分子に基づくが、この分子はショウジョウバエの胚の極性を決める遺伝子（セグメントポラリティー遺伝子）の1種である。ヘッジホッグは後期の胚発生や変態でも重要な役割を担っている。ー遺伝子）の1種である。ヘッジホッグは後期の胚発生や変態でも重要な役割を担っている。
, Hedgehog (v překladu „ježek“) je genová ro … Hedgehog (v překladu „ježek“) je genová rodina zahrnující u obratlovců tři geny, (Dhh), (Ihh) a (Shh). Geny této rodiny však mají i bezobratlí a nejčastěji se studují na octomilkách. Jejich produkty, signální proteiny, se účastní stejnojmenné signalizační kaskády hedgehog. Tyto bílkoviny obsahují navázaný cholesterol a mastnou kyselinu (jedná se tedy o lipoproteiny). Signalizace pomocí hedgehog proteinů má napříč živočišnou říší řadu funkcí. U octomilky řídí vývoj křídel, u ploštěnek regeneraci a u savců vývoj mozku, trávicí soustavy a prstů.vců vývoj mozku, trávicí soustavy a prstů.
, Hedgehog (en français : hérisson), est une … Hedgehog (en français : hérisson), est une protéine. Elle est présente chez les invertébrés (c'est une protéine de polarité segmentaire, impliquée dans le contrôle des patrons de développement embryonnaire). Il y a 3 gènes homologue du gène Hedgehog chez les vertébrés. La voie de signalisation de Hegdehog est complexe et fait intervenir plusieurs autres protéines. Patched et Wingless, notamment, sont aussi nécessaires à la segmentation et à la différenciation et permettent la régulation de l’expression de Hedgehog.la régulation de l’expression de Hedgehog.
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| rdfs:label |
ヘッジホッグシグナル伝達経路
, Hedgehog
, Via de sinalização Hedgehog
, Hedgehog (genetika)
, Hedgehog signaling pathway
, Hedgehog-Signalweg
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