Huntington's Disease

Definition: Huntington's Disease

Huntington's Disease

Noun

1. Hereditary; develops in adulthood and ends in dementia.

Domain	Definitions
Medicine	A fatal neurodegenerative disease. Source: European Union. (references)
	A degenerative neuropsychiatric disorder, characteristically affecting the basal ganglia but also affecting widespread areas of the central nervous system. Source: European Union. (references)
Source: compiled by the editor from various references; see credits.

Huntington's disease or Huntington's chorea is an inherited disorder characterized by abnormal body movements called chorea and loss of memory. The incidence is about 1/200,000. The causative gene (which is one of the first identified to cause an inherited disease) is located on chromosome 4. The product of this gene is a protein called huntingtin. The continuous accumulation of huntingtin neuronal cells gives rise to cell death, especially in the frontal lobes and the basal ganglia (mainly in the caudate nucleus) by some unknown mechanism. Huntingtin has a characteristic sequence of 40 or fewer glutamine residues in the normal form; the mutated huntingtin causing the disease has more than 40 residues.

Symptoms of Huntington's disease onset increasingly early the more glutamines a person carries within the repeating portion of their mutant huntingtin proteins. This number increases as the disease gene is passed on, so that the age of onset decreases with successive generations (although not infintely early, since patients with childhood symptoms tend not to have children themselves). Currently most Huntington's patients start to show symptoms in the 4th decade of life. These symptoms include the loss of cognitive abilities, changes in personality, quick jerking movements of face and body (i.e. chorea) and unsteadiness of gait. The diagnosis is established by neurological examination findings and the demonstration of cell loss, especially in the caudate nucleus, supported by a cranial CT or MRI scan findings. Although dopamine receptor blockers may have restricted benefits, there is no definite treatment for disease.

Unlike many hereditary illnesses, the alleles that cause Huntington's disease are dominant--meaning that having one parent with the disease guarantees offspring a 50% chance of inheriting it--and disease alleles are fatal in 100% of individuals that inherit them. Genetic testing can identify these individuals even before symptoms are present.

Because of these aspects, Huntingtion's raises many of the most pressing issues of bioethics. Disease carriers must contemplate whether to have children. Children of carriers must contemplate whether to have themselves genetically tested, despite absence of a cure and the possibile loss of health insurance as a result.

While theories as to how how the mutation brings about disease remain diverse and speculative, researchers have identified many specific subcellular abnormalities associated with the mutant protein, as well as unusual properties of the protein in vitro. Just as one example, in 2001, Max Perutz discovered that the glutamine residues form a nanotube¹ in vitro, and the mutated forms are long enough in principle to pierce cell membranes.

Literature :
¹ Proceedings, Volume 99, 5591-5596

Synonym: Huntington's Disease

Synonym: Huntington's chorea (n). (additional references)

Domain	Title
References	The Official Patient's Sourcebook on Huntington's Disease (reference) (more reference examples)

Books	Cell Transplantation for Huntington's Disease (Medical Intelligence Unit) (reference) Huntington's disease (reference) Living With Huntington's Disease (reference) (more book examples)
Source: compiled by the editor from various references; see credits.

Thumbnail	Description & Credit
	Recent Studies of Huntington's Disease : Marjorie Guthrie Lecture in Genetics / sponsored by [NINCDS] and [NIGMS]. Credit: National Library of Medicine.
Source: pictures compiled by the editor from various references; see picture credits.

Subject	Topic	Quote
Health		Huntington's disease is a hereditary, progressive and fatal neurological disorder. (references)
		Rosenblatt A, Leroi I. Neuropsychiatry of Huntington's disease and other basal ganglia disorders. (references)
		Diseases such as cystic fibrosis, muscular dystrophy, and Huntington's disease are single-gene disorders. (references)
Source: compiled by the editor from ICON Group International, Inc.; see credits.

Language		Translations for "Huntington's disease"; alternative meanings/domain in parentheses.
Danish		Huntingtons chorea (Huntington's chorea), præsenil demens med bevægelsesforstyrrelser (Huntington's chorea). (various references)

Dutch		ziekte van Huntington (Huntington's chorea). (various references)

Finnish		Huntingtonin tauti (Huntington's chorea). (various references)

French		maladie de Huntington (Huntington's chorea). (various references)

Greek		νόσος Huntington (Huntington's chorea). (various references)

Italian		malattia di Huntington (Huntington's chorea), corea degenerativa cronica (Huntington's chorea). (various references)

Pig Latin		untington'shay iseaseday

Spanish		enfermedad de Huntington, corea de Huntington (Huntington's chorea). (various references)
Source: compiled by the editor from various translation references.

Scrabble® Enable2K-Verified Anagrams
	Words within the letters "'-a-d-e-e-g-h-i-i-n-n-n-o-s-s-s-t-t-u"
	-5 letters: indigenousness.
Source: compiled by the editor from various references; see credits. SCRABBLE® is a registered trademark. All intellectual property rights in and to the game are owned in the U.S.A and Canada by Hasbro Inc., and throughout the rest of the world by J.W. Spear & Sons Limited of Maidenhead, Berkshire, England, a subsidiary of Mattel Inc. Mattel and Spear are not affiliated with Hasbro.

Hexadecimal (or equivalents, 770AD-1900s) (references)

48 75 6E 74 69 6E 67 74 6F 6E 27 73 44 69 73 65 61 73 65

Leonardo da Vinci (1452-1519; backwards) (references)

Binary Code (1918-1938, probably earlier) (references)

01001000 01110101 01101110 01110100 01101001 01101110 01100111 01110100 01101111 01101110 00100111 01110011 00100000 01000100 01101001 01110011 01100101 01100001 01110011 01100101

HTML Code (1990) (references)

&#72 &#117 &#110 &#116 &#105 &#110 &#103 &#116 &#111 &#110 &#39 &#115 &#32 &#68 &#105 &#115 &#101 &#97 &#115 &#101

ISO 10646 (1991-1993) (references)

0048 0075 006E 0074 0069 006E 0067 0074 006F 006E 0027 0073 0044 0069 0073 0065 0061 0073 0065

Encryption (beginner's substitution cypher): (references)

42878086758073868180985238758571678571

Note to the press & webmasters - this dictionary can be linked, indexed, or referred to using the following non-English expressions:
woordeboek, fjalor, ‏معجم, ‏قاموس, diccionariu, речник, diccionari, diksyonario, diksinario, 字典, gérlyver, slovník, ordbog, woordenboek, shimiyuc p'anca, orðabók, orðbók, dictionnaire, wurdboek, wörterbuch, λεξικό, אוצר מילים, szótár, uqausiit tukingit, dizionario, 字引 , じい, じびき, じて�", ディクショナリー , じり�", じしょ, �'�"かい, ディクショナリ , 사� �, dizionari, recnik, fockleyr, dikshonario, słownik, dicionário, dicţionar, dicziunari, словарь, lolomi fefiloi, foclair, abardair, faclair, briathrachan, pukuntau, leksikon, rečnik, vocabbulariu, diccionario, sí-chazamagâma, ordbok, lexikon, พจนานุกรม, sözlük, ansiklopedik sözlük, словник, довідник, có tính chất sách vở, geirlyfr, geiriadur, for dictionary;
definisie, qartësi, përcaktim, saktësi, ‏الوضوحية في الشيء, ‏حد, ‏تحديد, ‏تعريف, ‏التحديد, ‏الإيضاحية, яснота, сила, очертания, дефиниция, 定義 , 定义, definice, deskriptordefinition, definitie, määritelmä, définition, ορισμός, �"�'�"ר�", �"�'בל�", meghatározás, definíció, definizione, 確定 , ディーゼル電気車 , デ�'ドロ酢酸 , デフィニション , ディフィニション , ていぎ, かくてい, � �의, geyrid, meenaghey, keeayllaght, baght, definishon, definição, definiţie, determinare, definire, определение, definicija, definición, definition, açıklama, belirleme, belirtme, kesinleştirme, tanım, tarif, seçiklik, tanımlama, чіткість, тлумачення, виразність, визначення, дефініція, ясність, чітка чутність, sự định rõ, sự định nghĩa, lời định nghĩa sự định, diffiniad, darnodiad, for definition;
vertaling, transferim, transmetim, ‏ترجمة من لغة أجنبية للغة الأم, ‏ترجمة, ‏إفتتان, транслация, огъване, превод, предаване, поддаване, тълкуване, превеждане, 翻译, překlad, oversættelse, translatie, taajuusmuutos, translaatio, traduction, oersetting, Übersetzung, μετάφραση, תור�'מ� ות, תר�'ום, �"עתק�", �"עתק, fordítás, traduzione, 翻訳 , へい�"ういどう, やくしょ, やくしゅつ, �"うどく, ほ�"やく, トランスレーション , やくじゅつ, ほ�"やくしょ, 번역, tradukshon, tradução, translaţie, tãlmãcire, traducere, сдвиг, трансляция, перемещение, перевод, tumačenje, traducción, översättning, tercüme, процес перекладу, переклад, пояснення, переміщення, sự dịch, sự biến th� nh sự giải thích, trosiad, for translation;
Deens, danisht, danishte, ‏لغة الدانمركية, ‏نوع كعك, ‏دانماركي, датски език, датски, Daniko, 丹麦语, dánský, dánština, danskur, danskt, tanskalainen, danois, Deensk, dänisch, δανικόσ, δανόσ, עו�'ת שמרים, �"� י, dán, danska, Danmhairgis, danese, 덴마크, Danvargish, Danvargagh, danes, dinamarquês, danez, датский, danski, danski jezik, danés, dansk, danimarkalı, danimarka dili, датський, датська мова, tiếng Đan-mạch, for Danish;
Nederlands, Hollands, holandez, ‏هولندي, ‏اللغة الهولندية, холандски, немски език, холандски език, холандците, немски, Olandes, 菏蘭語 , 荷兰语, holandský, nizozemský, hollandsk, hollendskt, hollantilainen, néerlandais, Nederlânsk, holländisch, ολλανδικόσ, ολλανδόσ, holandisht, �"ול� �"י, holland, hollenskur, Ollainnis, olandese, 네덜란�"�, Belanda, Ollanish, Germaanish, Tatimana, nederlandsk, ulandes, hulandes, holandês, neerlandés, olandez, nemţesc, limba olandezã, german, голландский, holanđanin, u škripcu, holandski, holandés, bakratongo, holländsk, ชาวเนเธอร์แลน�"์, เกี่ยวกับเนเธอร์แลน�"์, � รรยา, alman, eş, flemenkçe, holandaca, hollanda, karı, hollandalı, hollandalılara özgü olan, Hollandali, hollanda'ya ait, голландська мова, голландський, ngôn ngữ khó hiểu, "b� xã", for Dutch;
Fins, finlandez, finlandishte, finlandisht, ‏اللغة الفنلندية, ‏فنلندية, ‏فنلندي, фински език, фински, Pinlandino, 芬蘭語 , 芬兰语, finský, finskt, suomi, suomalainen, finnois, Finlandaise, finlandais, finnisch, φινλανδικόσ, פי� י, finn, finnskur, finnska, finlandese, 핀란�"�, Fynlannish, Fynlannagh, finlandês, finês, finlandezã, финский, Finisi, finski jezik, finski, finlandés, finés, finsk, fince, finlandiya'ya özgü, фінська мова, фінський, tiếng Phần-lan, for Finnish;
Franse taal, Frans, franceze, francez, frëngjisht, frëng, frëngjishte, ‏فرنسي, ‏اللغة الفرنسية, ‏الشعب الفرنسي, gall, френски език, френски, Pranses, 法國 , 法文 , 法語 , 法语, francouzština, francouzský, franskur, franskt, ranskalainen, français, Frânsk, französisch, γάλλοσ, γαλλικόσ, γαλλική γλώσσα, γαλλίδα, צרפתי, צרפתית, francia, Fraincis, francese, フレコン化 , フランス�" , 仏文 , 仏 , ふつぶ�", フレンチ , フランセ , ふつ, �"�랑스, Perancis, Ny Frangee, Mooinjey ny Frank, frances, franses, francês, francezii, francezã, franţuzesc, franţuzeşte, французский, Falani, francuski jezik, francuski, francuzi, francés, sí-Fulentji, fransk, franska, fransızca, Fransiz, fransızca ile ilgili, fransız, fransa ile ilgili, французька мова, французький, Ffrengig, isiFulentshi, for French;
Grieks, Griek, ‏الإغريقي, ‏يوناني, ‏اللغة اليونانية, Griegu, гръцки език, гръцки, грък, Griyego, 希臘語 , 希腊语, řecký, řeètina, řek, græker, grikst, kreikkalainen, grec, Gryk, Gryksk, Gryks, grieche, ελληνικόσ, 'Ελληνας, יו� ית, יו� י, görög, Grikki, greco, ギリシア語 , ギリシア�", 그리스, Greagish, Greagagh, grego, grèc, greacã, греческий, Eleni, grk, grčki jezik, grčki, griego, grek, Yunanli, yunanlı, yunanca, yunan, Rumca, yunanistan'a ait, rum, грек, гречанка, грецька мова, грецький, kẻ cắp b� gi� gặp nhau, quân bạc bịp tôi không thể hiểu được điều đó thật l� kỳ phùng địch thủ, người Hy-lạp tiếng Hy-lạp kẻ bịp bợm, kẻ lừa đảo, Groegwr, for Greek;
Italianer, Italiaans, Italiaan, ‏شخص إيطالي, ‏اللغة الإيطالية, ‏الإيطالي, ‏إيطالي, Italianu, италиански език, италиански, италианец, Italyano, 意大利 , 意大利語 , 意大利语, italština, italský, ital, italiener, italienskt, italialainen, Italien, Italjaansk, italienisch, Ιταλός, italisht, איטלקי, איטלקית, olasz, Ítali, IodÚilis, italiano, 이탈리아, Iddaalish, Włoch, italianã, italienesc, italieneşte, italian, итальянский язык, итальянский, итальянец, Italia, italijanski, italijanski jezik, italijan, sí-Taliyáne, italienare, italiensk, italienska, เกี่ยวกับอิตาลี, ชาวอิตาลี, � าษาอิตาลี, italyanca, italyan, італі�"ць, італійська мова, італійський, італійка, for Italian;
Spaans, Spaanse taal, spanjoll, ‏اللغة الأسبانية, ‏الأسبانية, ‏أسباني, испански език, испански, espanyoles, Espanyol, 西班牙语, 西班牙文 , 西班牙語 , španìlský, španìlština, spanskt, espanjalainen, espagnol, Spaansk, spanisch, ισπανικά, ισπανικόσ, ισπανοί, karaiñe'êmegua, ספר�"ית, ספר�"י, spanyol, SpÚinnis, spagnolo, スペイン語 , スパイ罪 , スペイン�", スパニッシュ , 스페인, Spaainagh, Spaainish, spañó, espanhol, espanhòl, spaniolesc, spanioleşte, spaniol, испанский, Sipaniolo, španski jezik, španski, español, spanska språk, spansk, ispanyollar, ispanyolca, ispanyol, іспанська мова, іспанський, for Spanish;

Huntington's Disease

Definition: Huntington's Disease