肝臓型グリコーゲンホスホリラーゼ

WordNet

develop into a distinctive entity; "our plans began to take shape" (同)take form, take shape, spring
assume a form or shape; "the water formed little beads"
the visual appearance of something or someone; "the delicate cast of his features" (同)shape, cast
a mold for setting concrete; "they built elaborate forms for pouring the foundation"
a particular mode in which something is manifested; "his resentment took the form of extreme hostility"
a perceptual structure; "the composition presents problems for students of musical form"; "a visual pattern must include not only objects but the spaces between them" (同)shape, pattern
(biology) a group of organisms within a species that differ in trivial ways from similar groups; "a new strain of microorganisms" (同)variant, strain, var.
the phonological or orthographic sound or appearance of a word that can be used to describe or identify something; "the inflected forms of a word can be represented by a stem and a list of inflections to be attached" (同)word form, signifier, descriptor
a printed document with spaces in which to write; "he filled out his tax form"
an ability to perform well; "he was at the top of his form"; "the team was off form last night"
an arrangement of the elements in a composition or discourse; "the essay was in the form of a dialogue"; "he first sketches the plot in outline form"
to compose or represent:"This wall forms the background of the stage setting"; "The branches made a roof"; "This makes a fine introduction" (同)constitute, make
create (as an entity); "social groups form everywhere"; "They formed a company" (同)organize, organise
pursue a positive and satisfying existence; "You must accept yourself and others if you really want to live"
actually being performed at the time of hearing or viewing; "a live television program"; "brought to you live from Lincoln Center"; "live entertainment involves performers actually in the physical presence of a live audience" (同)unrecorded
abounding with life and energy; "the club members are a really live bunch"
charged with an explosive; "live ammunition"; "a live bomb"
exerting force or containing energy; "live coals"; "tossed a live cigarette out the window"; "got a shock from a live wire"; "live ore is unmined ore"; "a live bomb"; "a live ball is one in play"
highly reverberant; "a live concert hall"
in current use or ready for use; "live copy is ready to be set in type or already set but not yet proofread"
lead a certain kind of life; live in a certain style; "we had to live frugally after the war"
not recorded; "the opera was broadcast live"
of current relevance; "a live issue"; "still a live option"
a person who has a special life style; "a high liver"
large and complicated reddish-brown glandular organ located in the upper right portion of the abdominal cavity; secretes bile and functions in metabolism of protein and carbohydrate and fat; synthesizes substances involved in the clotting of the blood; sy
liver of an animal used as meat
someone who lives in a place; "a liver in cities"
one form in which body fuel is stored; stored primarily in the liver and broken down into glucose when needed by the body (同)animal_starch

PrepTutorEJDIC

〈C〉〈U〉(色・材質に対して)『形』,形状,姿 / 〈C〉『人体』,体つき / 〈C〉〈U〉『外観』,格好,人影 / 〈C〉〈U〉(ものの現れる)『形態』,形式 / 〈C〉(生物などの)『種類』,品種 / 〈U〉(芸術作品の)表現形式 / 〈U〉(特にスポーツで)(競技者の)フォーム / 〈U〉(心身の)調子,元気 / 〈C〉(印刷された)書き込み用紙,ひな形 / 〈U〉《話》(ある集団などの)やり方,仕方,方式 / 〈C〉〈U〉しきたり,伝統的な儀式 / 〈C〉(英国の学校・米国のある学校で)学年,学級 / 〈U〉〈C〉(意味に対する)形態,形;語形 / 〈C〉(通例寄りかかりのない)長い木のいす / 〈素材〉‘に'形を与える,(…に)…‘を'形作る《+『名』+『into』+『名』》 / (素材から)…‘を'形作る《+『名』+『out of』+『名』》 / …‘を'組織する,構成する / 〈考えなど〉‘を'まとめる,思い付く;〈計画など〉‘を'立てる / 〈習慣など〉‘を'身に付ける;〈交際など〉‘を'結ぶ / 〈文章など〉‘を'組み立てる;〈複数形など〉‘を'作る / (ある順序に)配列する,整列させる / 〈物が〉形を成す / 生じる,現れる / (…に)なる《+『into』+『名』》
『生きている』,生命がある / 《副詞[句]を伴って》〈人などが〉『生き続ける』 / 〈物事が〉長く続く,存続する / 《場所を表す副詞[句]を伴って》〈人などが〉『住む』,居住する / 《副詞[句]を伴って》『生活する』,暮らす / 生人を楽しむ,おもしろく桟らす / 《a+形容詞癌lifeを目的語にして》〈…を生活〉‘を'する / 〈思想など〉‘を'実銭する
《名詞の前にのみ用いて》『生きている』 / 『活気のある』 / (問題などが)当面の / 燃えている / (弾丸などが)まだ爆発してない,未発の / まだ動いている(働いている),有効な / 電流が通じている / (放走が)生の,実況の
《時間・距離》『…の間』 / 《利益・貢献》『…のために』 / 《適応・用途》『…向きの(に);』…用の(に);…のための(に) / 《目的・意向》『…のために,』に / 《敬意・記念》『…のために;』…を記念して / 《獲得・追求》『…を得るために』,を求めて / 《行く先・方向》『…あてに;』…[方面]行きの,に向けて / 《交換・報償など》『…に対して』;…と引き換えに;…のお返しに / 《原因・理由》『…のために』,なので / 《代理・代表》『…の代わりに;』…を代表して / 《形容詞の後に用いて》『…にとって,』には / 《比較級の後に用いて》『…の結果として,』…のために / 《対比》『…としては,』のわりには / 《関連》『…について,』の点では / 《資格・属性》『…として』 / 《賛成・支持》…『に賛成して』,を支持して / 《傾向・好み》…『に対する』,には / 《模倣》《おもに米》《英文》…の名を取って(after) / 《割合》…に対して / 《『for』+『名』〈人〉+『to』 doの形で,〈人〉が不定詞to doの意味上の主語を表して》…が(…することは,…するために) / […というのは]…だから
(ある態度で)生活する人;居住者
〈C〉『肝臓』 / 〈U〉(食用にする)動物の肝臓,レバー
グリコーゲン,糖原

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 肝ホスホリラーゼ欠損症（糖原病VI型、エール病） liver phosphorylase deficiency glycogen storage disease vi hers disease
2. ホスホリラーゼbキナーゼ欠損症 phosphorylase b kinase deficiency
3. Causes of hypoglycemia in infants and children
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. グリコーゲン脱分枝酵素欠損症（糖原病III型） glycogen debrancher deficiency glycogen storage disease iii

English Journal

Glycogen storage in the human retinal pigment epithelium: a comparative study of diabetic and non-diabetic donors.

Hernández C, Garcia-Ramírez M, García-Rocha M, Saez-López C, Valverde AM, Guinovart JJ, Simó R.Author information Diabetes and Metabolism Research Unit, Vall d'Hebron Research Institute, Universitat Autònoma de Barcelona, Pg. Vall d'Hebron 119-129, 08035, Barcelona, Spain, cristina.hernandez@vhir.org.AbstractLiver and muscle glycogen content is reduced in diabetic patients but there is no information on the effect of diabetes on the glycogen content in the retinal pigment epithelium (RPE). The main aim of the study was to compare the glycogen content in the RPE between diabetic and non-diabetic human donors. Glycogen synthase (GS) and glycogen phosphorylase (GP), the key enzymes of glycogen metabolism, as well as their isoforms, were also assessed. For this purpose, 44 human postmortem eye cups were included (22 from 11 type 2 diabetic and 22 from 11 non-diabetic donors matched by age). Human RPE cells cultured in normoglycemic and hyperglycemic conditions were also analyzed. Glycogen content as well as the mRNA, protein content and enzyme activity of GS and GP were determined. In addition, GS and GP isoforms were characterized. In the RPE from diabetic donors, as well as in RPE cells grown in hyperglycemic conditions, the glycogen content was increased. The increase in glycogen content was associated with an increase in GS without changes in GP levels. In RPE form human donors, the muscle GS isoform but not the liver GS isoform was detected. Regarding GP, the muscle and brain isoform of GP but not the liver GP isoform were detected. We conclude that glycogen storage is increased in the RPE of diabetic patients, and it is associated with an increase in GS activity. Further studies aimed at determining the role of glycogen deposits in the pathogenesis of diabetic retinopathy are warranted.
Acta diabetologica.Acta Diabetol.2014 Jan 24. [Epub ahead of print]
Liver and muscle glycogen content is reduced in diabetic patients but there is no information on the effect of diabetes on the glycogen content in the retinal pigment epithelium (RPE). The main aim of the study was to compare the glycogen content in the RPE between diabetic and non-diabetic human do
PMID 24458975

Novel PHKG2 mutation causing GSD IX with prominent liver disease: report of three cases and review of literature.

Albash B, Imtiaz F, Al-Zaidan H, Al-Manea H, Banemai M, Allam R, Al-Suheel A, Al-Owain M.Author information Medical Genetics Centre, Kuwait City, Kuwait.AbstractGlycogen storage disease type IX (GSD IX) is a common form of glycogenosis due to mutations in PHKA1, PHKA2, or PHKB and PHKG2 genes resulting in the deficiency of phosphorylase kinase. The first two genes are X-linked while the latter two follow an autosomal recessive inheritance. The majority of cases of GSD IX are attributed to defects in PHKA2 which usually cause a mild disease. We report three patients with PHKG2-related GSD IX presenting with significant hepatic involvement, fibrosis, and cirrhosis. Interestingly, the homozygosity mapping resolved a dilemma about an erroneously normal phosphorylase kinase activity in patient 1. The novel mutation found in all the three patients (p.G220E) affects the catalytic subunit of the phosphorylase kinase. Increasing evidence shows that patients with PHKG2 mutations have a severe hepatic phenotype within the heterogeneous GSD IX disorder. Therefore, defect in PHKG2 should be considered in patients with suspected glycogenosis associated with significant liver fibrosis and cirrhosis.
European journal of pediatrics.Eur J Pediatr.2013 Dec 11. [Epub ahead of print]
Glycogen storage disease type IX (GSD IX) is a common form of glycogenosis due to mutations in PHKA1, PHKA2, or PHKB and PHKG2 genes resulting in the deficiency of phosphorylase kinase. The first two genes are X-linked while the latter two follow an autosomal recessive inheritance. The majority of c
PMID 24326380

Hepatic glucose metabolism in late pregnancy: normal versus high-fat and -fructose diet.

Coate KC, Smith MS, Shiota M, Irimia JM, Roach PJ, Farmer B, Williams PE, Moore MC.Author information Department of Molecular Physiology and Biophysics, Vanderbilt University School of Medicine, Nashville, Tennessee, USA.AbstractNet hepatic glucose uptake (NHGU) is an important contributor to postprandial glycemic control. We hypothesized that NHGU is reduced during normal pregnancy and in a pregnant diet-induced model of impaired glucose intolerance/gestational diabetes mellitus (IGT/GDM). Dogs (n = 7 per group) that were nonpregnant (N), normal pregnant (P), or pregnant with IGT/GDM (pregnant dogs fed a high-fat and -fructose diet [P-HFF]) underwent a hyperinsulinemic-hyperglycemic clamp with intraportal glucose infusion. Clamp period insulin, glucagon, and glucose concentrations and hepatic glucose loads did not differ among groups. The N dogs reached near-maximal NHGU rates within 30 min; mean ± SEM NHGU was 105 ± 9 µmol·100 g liver⁻¹·min⁻¹. The P and P-HFF dogs reached maximal NHGU in 90-120 min; their NHGU was blunted (68 ± 9 and 16 ± 17 µmol·100 g liver⁻¹·min⁻¹, respectively). Hepatic glycogen synthesis was reduced 20% in P versus N and 40% in P-HFF versus P dogs. This was associated with a reduction (>70%) in glycogen synthase activity in P-HFF versus P and increased glycogen phosphorylase (GP) activity in both P (1.7-fold greater than N) and P-HFF (1.8-fold greater than P) dogs. Thus, NHGU under conditions mimicking the postprandial state is delayed and suppressed in normal pregnancy, with concomitant reduction in glycogen storage. NHGU is further blunted in IGT/GDM. This likely contributes to postprandial hyperglycemia during pregnancy, with potential adverse outcomes for the fetus and mother.
Diabetes.Diabetes.2013 Mar;62(3):753-61. doi: 10.2337/db12-0875. Epub 2012 Dec 6.
Net hepatic glucose uptake (NHGU) is an important contributor to postprandial glycemic control. We hypothesized that NHGU is reduced during normal pregnancy and in a pregnant diet-induced model of impaired glucose intolerance/gestational diabetes mellitus (IGT/GDM). Dogs (n = 7 per group) that were
PMID 23223020

Japanese Journal

HEPATIC GLYCOGEN METABOLISM IN NORMAL DEVELOPING AND INTRAUTERINE GROWTH-RETARDED RAT FETUSES

CHENG Kuang-Ming
日本産科婦人科學會雜誌 40(6), 781-788, 1988-06-01
… 正常発育とIUGR胎仔の肝glycogenとglycogen代謝における合成律速酵素であるsynthaseおよび分解律速酵素であるphosphorylaseの活性を経日的に比較検討した. … また, 組織染色法を用いて光顕および電顕レベルにおける肝細胞内glycogenの組織局在の検索を加えた. …
NAID 110002113161

ラット子宮内発育遅延仔における肝臓glycogenの代謝調節

小川隆吉
日本産科婦人科學會雜誌 37(9), 1841-1848, 1985-09-01
… 妊娠ラットを用い,子宮動静脈結紮によりIUGRを作成し,妊娠19日,20日両日にDexamethasone(DXM)を経母獣的に投与,妊娠20日に開腹して胎仔をとりだし,胎仔肝glycogen量,glycogen synthetase活性およびglycogen phosphorylase活性を測定し以下の成績をえた. …
NAID 110002221317

Related Pictures

★リンクテーブル★

リンク元	「肝臓型グリコーゲンホスホリラーゼ」
関連記事	「form」「live」「glycogen phosphorylase」「liver」「glycogen」