鉄代謝障害
WordNet
- press and smooth with a heated iron; "press your shirts"; "she stood there ironing" (同)iron_out, press
- a heavy ductile magnetic metallic element; is silver-white in pure form but readily rusts; used in construction and tools and armament; plays a role in the transport of oxygen by the blood (同)Fe, atomic number 26
- implement used to brand live stock (同)branding iron
- home appliance consisting of a flat metal base that is heated and used to smooth cloth (同)smoothing iron
- a golf club that has a relatively narrow metal head
- bring disorder to (同)disarray
- a physical condition in which there is a disturbance of normal functioning; "the doctor prescribed some medicine for the disorder"; "everyone gets stomach upsets from time to time" (同)upset
- a disturbance of the peace or of public order
- garments (clothes or linens) that are to be (or have been) ironed; "there was a basketful of ironing to do"
- the work of using heat to smooth washed clothes in order to remove any wrinkles
- the organic processes (in a cell or organism) that are necessary for life (同)metabolic_process
- (of linens or clothes) smoothed with a hot iron
PrepTutorEJDIC
- 〈U〉『鉄』 / 〈U〉鉄のように堅い(強い,冷たい)こと / 〈C〉鉄製の器具 / 〈C〉[電気]『アイロン』 / 〈C〉アイアン(球を打つ部分が金属のゴルフクラブ) / 〈C〉《複数形で》手かせ,足かせ / 《名詞の前にのみ用いて》『鉄の』,鉄製の / 鉄のように堅い(強い,冷たい) / …‘に'アイロンをかける / 〈人が〉アイロンをかける
- 〈U〉『無秩序』,混乱,乱雑(confusion) / 《しばしば複数形で》(社会的・政治的な)粉争,騒動 / 〈C〉(肉体的・精神的な)不調,異常,障害 / …‘の'秩序を乱す / 〈心身〉‘に'異常を起こさせる
- アイロンかけ / アイロンかけをした(をする)衣類
- 新陳代謝,物質交代
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/11/30 12:15:32」(JST)
[Wiki en表示]
Iron metabolism disorder |
Classification and external resources |
ICD-10 |
E83.1 |
ICD-9 |
275.0 |
MeSH |
D019189 |
Genes involved in iron metabolism disorders include HFE and TFR2.[1]
Hepcidin is the master regulator of iron metabolism and, therefore, most genetic forms of iron overload can be thought of as relative hepcidin deficiency in one way or another[1]. For instance, a severe form of iron overload, juvenile hemochromatosis, is a result of severe hepcidin deficiency. The majority of cases are caused by mutations in the hemojuvelin gene (HJV or RGMc/repulsive guidance molecule c). The exceptions, people who have mutations in the gene for ferroportin, prove the rule: these people have plenty of hepcidin, but their cells lack the proper response to it. So, in people with ferroportin proteins that transport iron out of cells without responding to hepcidin's signals to stop, they have a deficiency in the action of hepcidin, if not in hepcidin itself.
But the exact mechanisms of most of the various forms of adult hemochromatosis, which make up most of the genetic iron overload disorders, remain unsolved. So while researchers have been able to identify genetic mutations causing several adult variants of hemochromatosis, they now must turn their attention to the normal function of these mutated genes.
These genes represent multiple steps along the pathway of iron regulation, from the body's ability to sense iron, to the body's ability to regulate uptake and storage. Working out the functions of each gene in this pathway will be an important tool for finding new methods of treating genetic disorders, as well as for understanding the basic workings of the pathway.
So though many mysteries of iron metabolism remain, the discovery of hepcidin already allows a much better understanding of the nature of iron regulation, and makes researchers optimistic that many more breakthroughs in this field are soon to come.
See also[edit]
- Human iron metabolism
- Iron deficiency anemia
References[edit]
- ^ Roy CN, Andrews NC (October 2001). "Recent advances in disorders of iron metabolism: mutations, mechanisms and modifiers". Hum. Mol. Genet. 10 (20): 2181–6. doi:10.1093/hmg/10.20.2181. PMID 11673399.
External links[edit]
- http://sickle.bwh.harvard.edu/menu_iron.html
- Andrews NC (December 1999). "Disorders of iron metabolism". N. Engl. J. Med. 341 (26): 1986–95. doi:10.1056/NEJM199912233412607. PMID 10607817.
Inborn error of metal metabolism (E83, 275)
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Transition metal |
Fe
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high: Primary iron overload disorder: Hemochromatosis/HFE1 · Juvenile/HFE2 · HFE3 · African iron overload/HFE4 · Aceruloplasminemia · Atransferrinemia · Hemosiderosis
deficiency: Iron deficiency
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Cu
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high: Copper toxicity · Wilson's disease
deficiency: Copper deficiency · Menkes disease/Occipital horn syndrome
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Zn
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high: Zinc toxicity
deficiency: Acrodermatitis enteropathica
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Electrolyte |
Na+ and K+
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see Template:Water-electrolyte imbalance and acid-base imbalance
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PO43−
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high: Hyperphosphatemia
deficiency: Hypophosphatemia · alkaline phosphatase (Hypophosphatasia)
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Mg2+
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high: Hypermagnesemia
deficiency: Hypomagnesemia
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Ca2+
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high: Hypercalcaemia · Milk-alkali syndrome (Burnett's) · Calcinosis (Calciphylaxis, Calcinosis cutis) · Calcification (Metastatic calcification, Dystrophic calcification) · Familial hypocalciuric hypercalcemia
deficiency: Hypocalcaemia · Osteomalacia · Pseudohypoparathyroidism (Albright's hereditary osteodystrophy) · Pseudopseudohypoparathyroidism
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noco, nuvi, sysi/epon, met
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UpToDate Contents
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English Journal
- Severe renal tubulopathy in a newborn due to BCS1L gene mutation: Effects of different treatment modalities on the clinical course.
- Ezgu F, Senaca S, Gunduz M, Tumer L, Hasanoglu A, Tiras U, Unsal R, Bakkaloglu SA.SourceDepartment of Pediatric Metabolism and Nutrition, Gazi University Hospital, Ankara, Turkey. Electronic address: fezgu@gazi.edu.tr.
- Gene.Gene.2013 Oct 10;528(2):364-6. doi: 10.1016/j.gene.2013.07.007. Epub 2013 Jul 26.
- Very early onset Toni-Debré-Fanconi Syndrome, a disorder of proximal renal tubules of the kidney which results in the increased urinary excretion of glucose, amino acids, uric acid, phosphate and bicarbonate, could be the manifestation of various inborn errors. Defects of oxidative phosphorylation
- PMID 23892085
- 6-Hydroxydopamine promotes iron traffic in primary cultured astrocytes.
- Zhang HY, Wang ND, Song N, Xu HM, Shi LM, Jiang H, Xie JX.SourceDepartment of Physiology, Shandong Provincial Key Laboratory of Pathogenesis and Prevention of Neurological Disorders and State Key Disciplines: Physiology, Medical College of Qingdao University, No. 308 Ningxia Road, Qingdao, 266071, China.
- Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine.Biometals.2013 Oct;26(5):705-14. doi: 10.1007/s10534-013-9647-x. Epub 2013 Jun 17.
- It is well known that disrupted brain iron homeostasis was involved in Parkinson's disease. We previously reported 6-hydroxydopamine (6-OHDA) could enhance iron influx and attenuate iron efflux process, thus promote iron accumulation in neurons. Astrocytes, the major glial cell type in the central n
- PMID 23771608
- Interplay between the pro-oxidant and antioxidant systems and proinflammatory cytokine levels, in relation to iron metabolism and the erythron in depression.
- Rybka J, Kędziora-Kornatowska K, Banaś-Leżańska P, Majsterek I, Carvalho LA, Cattaneo A, Anacker C, Kędziora J.SourceDepartment of Biochemistry, Collegium Medicum UMK, 85-092 Bydgoszcz, Poland. joanna.rybka@scienceventure.eu
- Free radical biology & medicine.Free Radic Biol Med.2013 Oct;63:187-94. doi: 10.1016/j.freeradbiomed.2013.05.019. Epub 2013 May 23.
- As there is strong evidence for inflammation and oxidative stress in depression, the aim of this study was to elucidate the relationship between oxidative imbalance and cellular immune response and to ask whether these processes are linked with iron metabolism in depressed patients. Blood was collec
- PMID 23707456
Japanese Journal
- Iron Deficiency and Severe Plasmodium falciparum Malaria
- Awah Nancy W.,Kaneko Akira
- Clinical Infectious Diseases 54(8), 1145-1147, 2012-04-15
- NAID 120004606861
- 新生児ヘモクロマトーシスと両側低形成腎を合併し,慢性腎不全に対して腹膜透析を施行した1症例
- 服部 希世子,仲里 仁史,河野 智康,佐藤 歩,田村 博,松本 志郎,市原 順子,辛嶋 真実,田仲 健一,岩井 正憲,伊藤 徳浩,西 一彦,三渕 浩,近藤 裕一,遠藤 文夫
- 日本小児腎臓病学会雑誌 24(1), 68-73, 2011
- 新生児ヘモクロマトーシスは新生児早期より肝臓を中心に鉄の沈着を生じ,重篤な肝障害を呈する稀な疾患で,肝障害が急速に進行し致死的になることが多い。 今回われわれは,新生児ヘモクロマトーシスを合併し,両側低形成腎による慢性腎不全に至った症例を経験した。小腸閉鎖,胎便性腹膜炎を認め,日齢1に小腸閉鎖根治術を施行したが術後より乏尿となり次第に腎障害が進行した。その後,肝障害と高フェリチン血症が出現した。 …
- NAID 130001353980
- Excess Copper Chelating Therapy for Wilson Disease Induces Anemia and Liver Dysfunction
- Harada Masaru,Miyagawa Koichiro,Honma Yuichi,Hiura Masaaki,Shibata Michihiko,Matsuhashi Toru,Abe Shintaro,Harada Riko,Tabaru Akinari
- Internal Medicine 50(14), 1461-1464, 2011
- … Wilson disease is a genetic disorder characterized by accumulation of copper in the body. … Copper-binding ceruloplasmin is important for iron metabolism. … Excess copper chelating treatment-induced anemia and iron deposition in the liver was suspected. …
- NAID 130000969422
Related Links
- Genes involved in iron metabolism disorders include HFE and TFR2. Hepcidin is the master regulator of iron metabolism and, therefore, most genetic forms of iron overload can be thought of as relative hepcidin deficiency in one way or ...
Related Pictures
★リンクテーブル★
[★]
- 英
- iron metabolism disorder
[★]
- 障害:個人的苦痛や機能の障害があるので「疾病」とは言えるものの、その背景にある臓器障害がもう一つはっきりしない場合に用いられる。(PSY.9)
- an untidy state; a lack of order or organization (⇔order)
- violent behaviour of large groups of people
- an illness that cause a part of the body to stop functioning correctly
- disease <> illness <> disorder
- 乱す、乱雑にする。(人)の(心身の)調子を狂わせる。
[★]
鉄