WordNet
- make ones way by force; "He muscled his way into the office"
- animal tissue consisting predominantly of contractile cells (同)muscular_tissue
- one of the contractile organs of the body (同)musculus
- authority or power or force (especially when used in a coercive way); "the senators used their muscle to get the party leader to resign"
PrepTutorEJDIC
- 〈U〉(動物体の組織としての)『筋肉』,筋(きん);〈C〉(体の各部を動かす)筋肉 / 〈U〉力,(特に)筋力,腕力 / 《俗に》(…に)強引に割り込む《+『in on』(『into, through』)+『名』》
- 〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥
- 筋肉の,筋力のある / 力ずくの
UpToDate Contents
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- 1. 筋ホスホリラーゼ欠損症（糖原病V型、マッカードル病） muscle phosphorylase deficiency glycogen storage disease v mcardle disease
- 2. 横紋筋融解症の原因 causes of rhabdomyolysis
- 3. 神経筋疾患の評価における筋酵素 muscle enzymes in the evaluation of neuromuscular diseases
- 4. グルコースおよびグリコーゲン代謝の遺伝性疾患の概要 overview of inherited disorders of glucose and glycogen metabolism
- 5. ホスホグリセリン酸キナーゼ欠損症およびホスホグリセリン酸ムターゼ欠損症 phosphoglycerate kinase deficiency and phosphoglycerate mutase deficiency
English Journal
- Oxidative stress and Nrf2 signaling in McArdle disease.
- Kitaoka Y, Ogborn DI, Nilsson MI, Mocellin NJ, MacNeil LG, Tarnopolsky MA.Author information Departments of Pediatrics and Medicine, McMaster University, 1200 Main Street West, Hamilton, Ontario, L8N 3Z5 Canada. Electronic address: kitaoka@mcmaster.ca.AbstractMcArdle disease (MD) is a metabolic myopathy due to myophosphorylase deficiency, which leads to a severe limitation in the rate of adenosine triphosphate (ATP) resynthesis. Compensatory flux through the myoadenylate deaminase > > xanthine oxidase pathway should result in higher oxidative stress in skeletal muscle; however, oxidative stress and nuclear factor erythroid 2-related factor 2 (Nrf2) mediated antioxidant response cascade in MD patients have not yet been examined. We show that MD patients have elevated muscle protein carbonyls and 4-hydroxynonenal (4-HNE) in comparison with healthy, age and activity matched controls (P < 0.05). Nuclear abundance of Nrf2 and Nrf2-antioxidant response element (ARE) binding was also higher in MD patients compared with controls (P < 0.05). The expressions of Nrf2 target genes were also higher in MD patients vs. controls. These observations suggest that MD patients experience elevated levels of oxidative stress, and that the Nrf2-mediated antioxidant response cascade is up-regulated in skeletal muscle to compensate.
- Molecular genetics and metabolism.Mol Genet Metab.2013 Nov;110(3):297-302. doi: 10.1016/j.ymgme.2013.06.022. Epub 2013 Jul 6.
- McArdle disease (MD) is a metabolic myopathy due to myophosphorylase deficiency, which leads to a severe limitation in the rate of adenosine triphosphate (ATP) resynthesis. Compensatory flux through the myoadenylate deaminase > > xanthine oxidase pathway should result in higher oxidative stres
- PMID 23906480
- McArdle's disease: a clinical review and case report.
- Keel BR, Brit M.Author information Chattanooga Emergency Medicine, TN, USA. bradleykeel@gmail.comAbstractMcArdle's Disease is a rare glycogen disease involving deficiency in muscle phosphorylase. This deficiency can lead to rhabdomyolysis and subsequently renal failure. McArdle's Disease has a similar presentation as several other metabolic myopathies with exercise-induced fatigue, myalgias, weakness or unexplained rhabdomyolysis. Suspicion should be raised in the presence of unexplained symptoms, and muscle biopsy can be done to confirm the diagnosis.
- Tennessee medicine : journal of the Tennessee Medical Association.Tenn Med.2013 Nov-Dec;106(10):33, 37.
- McArdle's Disease is a rare glycogen disease involving deficiency in muscle phosphorylase. This deficiency can lead to rhabdomyolysis and subsequently renal failure. McArdle's Disease has a similar presentation as several other metabolic myopathies with exercise-induced fatigue, myalgias, weakness o
- PMID 24282836
- Phosphorylase re-expression, increase in the force of contraction and decreased fatigue following notexin-induced muscle damage and regeneration in the ovine model of McArdle disease.
- Howell JM, Walker KR, Creed KE, Dunton E, Davies L, Quinlivan R, Karpati G.Author information School of Veterinary and Life Sciences, Murdoch University, Perth 6150, Western Australia, Australia; Australian Neuro-Muscular Research Institute,CNND, University of Western Australia, Perth 6150, Western Australia, Australia. Electronic address: J.Howell@murdoch.edu.au.AbstractMcArdle disease is caused by a deficiency of myophosphorylase and currently a satisfactory treatment is not available. The injection of notexin into, or the layering of notexin onto, the muscles of affected sheep resulted in necrosis followed by regeneration of muscle fibres with the expression of both non-muscle isoforms of phosphorylase within the fibres and a reduction of the amount of glycogen in the muscle with an increase in the strength of contraction and a decrease in fatiguability in the muscle fibres. The sustained re-expression of both the brain and liver isoforms of phosphorylase within the muscle fibres provides further emphasis that strategies to enhance the re-expression of these isoforms should be investigated as a possible treatment for McArdle disease.
- Neuromuscular disorders : NMD.Neuromuscul Disord.2013 Oct 26. pii: S0960-8966(13)00983-8. doi: 10.1016/j.nmd.2013.10.003. [Epub ahead of print]
- McArdle disease is caused by a deficiency of myophosphorylase and currently a satisfactory treatment is not available. The injection of notexin into, or the layering of notexin onto, the muscles of affected sheep resulted in necrosis followed by regeneration of muscle fibres with the expression of b
- PMID 24309536
Japanese Journal
- Effect of vitamin B6 deficiency on the expression of glycogen phosphorylase mRNA in rat liver and skeletal muscle
- OKA T.
- Experientia 50, 127-129, 1994
- NAID 80007491616
- Effect of Vitamin B6 Deficiency on Glycogen Metabolism in the Skeletal Muscle, Heart, and Liver of Rats.
- OKADA Mitsuko,ISHIKAWA Kazumi,WATANABE Kayo
- Ｊｏｕｒｎａｌ ｏｆ Ｎｕｔｒｉｔｉｏｎａｌ Ｓｃｉｅｎｃｅ ａｎｄ Ｖｉｔａｍｉｎｏｌｏｇｙ 37(4), 1991
- … The effect of vitamin B6 deficiency was studied on glycogen metabolism in the gastrocnemius muscle, heart, and liver of rats. … The glycogen phosphorylase activities in the gastrocnemius muscle and heart but not in the liver of vitamin B6-deficient rats were significantly decreased. … The decrease in the enzyme activity in the gastrocnemius muscle was due to decrease in the amount of enzyme. …
- NAID 130001369195
Related Links
- Myophosphorylase (muscle phosphorylase) deficiency (MIM #232600), also known as McArdle disease, is the most common glycogen storage disease (GSD) affecting the muscle (). The GSDs are generally categorized by number ...
- Muscle phosphorylase deficiency symptoms, causes, diagnosis, and treatment information for Muscle phosphorylase deficiency (McArdle disease) with alternative diagnoses, full-text book chapters, misdiagnosis, research ...
Related Pictures
★リンクテーブル★
| リンク元 | 「糖原病」「マッカードル病」「glycogen storage disease type V」「MPD」「筋ホスホリラーゼ欠損症」 |
| 関連記事 | 「deficiency」「muscle」 |
「糖原病」
- 英
- glycogen storage disease GSD GSDs , glycogenosis
- 同
- グリコーゲン病、グリコーゲン蓄積病
- 関
- グリコーゲン、解糖系
- first aid step1 2006 p.98
定義
- グリコーゲン代謝系酵素欠損により、組織にグリコーゲンが蓄積する疾患群
分類 (FB.304,HIM.2458)
| 型 | 欠損酵素 | 組織 | グリコーゲン構造 | 病名 | 発症時期 | 症状 |
| I | グルコース-6-ホスファターゼ | 肝臓 | 正常 | フォン・ギールケ病 | childhood | growth retardation, enlarged liver and kidney, hypoglycemia, elevated blood lactate, cholesterol, triglycerides, and uric acid |
| II | α-1,4-グルコシダーゼ | リソソーム | 正常 | ポンペ病 | childhood | |
| III | アミロ-1,6-グルコシダーゼ debrancher enzyme deficiency |
全器官 | 最外層分子がないか非常に短い | コリ病 | childhood | hypoglycemia, hepatomegaly, short-stature, and myopathty |
| IV | アミロ-(1,4→1,6)-トランスグリコシラーゼ brancher enzyme deficiency |
肝臓、全器官? | 非常に長い枝無し鎖 | アンダーセン病 | infantile failure to thrive, cirrhosis, liver failure, and extreme hypotonia. | |
| V | グリコーゲンホスホリラーゼ muscle phosphorylase deficiency |
筋肉 | 正常 | マッカードル病 | adult | exercise intolerance, muscle cramps, myoglobinuria on strenuous exercise, increased CK |
| VI | グリコーゲンホスホリラーゼ hepatic phosphorylase deficiency |
肝臓 | 正常 | エルス病 | hematomegaly and variable hypoglycemia | |
| VII | 6-ホスホフルクト-1-キナーゼ | 筋肉 | 正常 | 垂井病 | ||
| VIII | ホスホリラーゼキナーゼ | 肝臓 | 正常 | X染色体ホスホリラーゼキナーゼ欠損症 | ||
| IX | ホスホリラーゼキナーゼ | 全器官 | 正常 | childhoog | ||
| 0 | グリコーゲンシンターゼ | 肝臓 | 正常だが少量 |
「マッカードル病」
- 英
- McArdle disease
- 英
- V型糖原病 type V glycogen storage disease, 糖原病V型 glycogen storage disease type V, glycogenosis type V
- McArdle病、筋ホスホリラーゼ欠損症 muscle phosphorylase deficiency MPD。
- 関
- 糖原病
- 同
- McArdle病
- first aid step1 2006 p.98
概念
- 筋型の糖原病に分類される
- 筋肉における先天的なグリコーゲンホスホリラーゼ欠損により、 グリコーゲン分解が困難となる｡運動時の疫撃、運動障害｡
病因
疫学
遺伝形式
病変形成＆病理
症状
- 激しい運動後が引き金となり疼痛、ミオグロビン尿を呈する。
診断
検査
治療
予後
予防
USMLE
- Q book p.139 33
「glycogen storage disease type V」
「MPD」
「筋ホスホリラーゼ欠損症」
「deficiency」
- n.
- 不足、欠乏、欠失、欠如、欠損、不十分。栄養不足、栄養素欠乏、欠乏症。(遺伝子)(染色体内の)遺伝子欠失
- 欠けているもの、不足している物。不足分。不完全なもの、欠点のあるもの
- 関
- absence, agenesis, dearth, defect, defective, deficient, deficit, delete, deletion, deletional, depletion, deprivation, deprive, lack, miss, missing, morphological defect, paucity, scarce, scarcity, starve
「muscle」
- n.