β-glucuronidase deficiency

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1. 〈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

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Japanese Journal

• Encapsulation Cell Therapy for Mucopolysaccharidosis Type VII Using Genetically Engineered Immortalized Human Amniotic Epithelial Cells

• Nakama Hideyuki,Ohsugi Keiko,Otsuki Taisuke,Date Isao,Kosuga Motomichi,Okuyama Torayuki,Sakuragawa Norio
• The Tohoku Journal of Experimental Medicine 209(1), 23-32, 2006
• … Mucopolysaccharidosis type VII (MPSVII) is a lysosomal storage disease resulted from a deficiency of the enzyme β-glucuronidase (GUSB), which is necessary for degradation of glycosaminoglycans (GAGs). … The deficiency of GUSB causes progressive accumulation of GAGs and subsequent lysosomal distension in multiple tissues, including the central nervous system (CNS). …
• NAID 130004459486

• Construction of Artificial Promoters Highly Responsive to Iron Deficiency

• Kobayashi Takanori,Nakayama Yuko,Takahashi Michiko [他],Inoue Haruhiko,Nakanishi Hiromi,Yoshihara Toshihiro,Mori Satoshi,Nishizawa Naoko K
• Soil science and plant nutrition 50(7), 1167-1175, 2004-12
• … Iron deficiency-responsive element 1 (IDE1) and IDE2 are cis-acting elements that are responsible for Fe-deficiency-inducible and root-specific expression of the barley (Hordeum vulgare L.) gene IDS2 (Fe-deficiency-specific clone no. 2). … Modules containing IDE1 and IDE2 of the IDS2 promoter were used as repeats or were linked to the Fe-deficiency-responsive promoter of barley IDS3, and were connected to known enhancer-like sequences. …
• NAID 10014191781

• Independent Roles of Methionine and O-Acetyl-L-Serine in the Regulation of the β Subunit Gene of β-Conglycinin

• Yokota Masami,Kim Hoyeun,Hayashi Hiroaki [他],Chino Mitsuo,Naito Satoshi,Fujiwara Toru
• Soil science and plant nutrition 48(1), 87-94, 2002-02
• … The gene encoding the β subunit of β-conglycinin, one of the major seed storage proteins of soybean (Glycine max [L.] Merr.), is downregulated by methionine (Met) and upregulated by O-acetyl-L-serine (OAS). … We examined the interaction between Met and OAS in the regulation of the expression of the β subunit gene in in vitro-cultured immature soybean cotyledons. …
• NAID 110001717608

Related Links

• Beta-glucuronidase - Wikipedia, the free encyclopedia

Deficiencies in β-glucuronidase result in the non recessive inherited metabolic disease known as Sly syndrome or Mucopolysaccharidosis VII. A deficiency in this enzyme results in the build-up of non-hydrolyzed mucopolysaccharides in the ...

• Mucopolysaccharidosis type VII - Genetics Home Reference

GAGs were originally called mucopolysaccharides, which is where this condition gets its name. Mutations in the GUSB gene reduce or completely eliminate the function of β-glucuronidase. The shortage (deficiency) of β-glucuronidase leads to ...

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★リンクテーブル★

リンク元	「ムコ多糖症VII型」
関連記事	「deficiency」

「ムコ多糖症VII型」

　　[★]

英

mucopolysaccharidosis VII, mucopolysaccharidosis type VII MPS VII

同

β-グルクロニダーゼ欠損症 β-glucuronidase deficiency、スライ症候群 Sly syndrome

関

スライ病

「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