protective protein/cathepsin A

同

PPCA

WordNet

1. showing care; "a protective mother"
2. (usually followed by `of'
3. intended or adapted to afford protection of some kind; "a protective covering"; "the use of protective masks and equipment"; "protective coatings"; "kept the drunken sailor in protective custody"; "animals with protective coloring"; "protective tariffs"
4. any of a large group of nitrogenous organic compounds that are essential constituents of living cells; consist of polymers of amino acids; essential in the diet of animals for growth and for repair of tissues; can be obtained from meat and eggs and milk and legumes; "a diet high in protein"
5. the 1st letter of the Roman alphabet (同)a
6. the blood group whose red cells carry the A antigen (同)type_A, group A

PrepTutorEJDIC

1. 保護の,保護する;(…を)保護する《+『toward』+『名』》
2. 蛋白(たんばく)質
3. answer / ampere
4. arsenicの化学記号

UpToDate Contents

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• 1. プロテインS欠乏症：臨床症状および診断 protein s deficiency clinical manifestations and diagnosis
• 2. 幼児の食物蛋白誘発性直腸炎/大腸炎および腸疾患 food protein induced proctitis colitis and enteropathy of infancy
• 3. プロテインC欠乏症：臨床症状および診断 protein c deficiency clinical manifestations and diagnosis
• 4. 食物蛋白誘発胃腸炎（新生児-乳児消化管アレルギー）（FPIES） food protein induced enterocolitis syndrome fpies
• 5. 第V因子ライデンおよび活性化プロテインC抵抗性：臨床症状および診断 factor v leiden and activated protein c resistance clinical manifestations and diagnosis

English Journal

• Chaperone-mediated gene therapy with recombinant AAV-PPCA in a new mouse model of type I sialidosis.

• Bonten EJ, Yogalingam G, Hu H, Gomero E, van de Vlekkert D, d'Azzo A.SourceDepartment of Genetics, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
• Biochimica et biophysica acta.Biochim Biophys Acta.2013 Oct;1832(10):1784-92. doi: 10.1016/j.bbadis.2013.06.002. Epub 2013 Jun 12.
• The lysosomal storage disease sialidosis is caused by a primary deficiency of the sialidase N-acetyl-α-neuraminidase-1 (NEU1). Patients with type I sialidosis develop an attenuated, non-neuropathic form of the disease also named cherry red spot myoclonus syndrome, with symptoms arising during juven
• PMID 23770387

• Ultrastructural change of ligamentum flavum in galactosialidosis.

• Nakama S, Hagiwara S, Kato M, Kanaya Y, Watanabe H.SourceDepartment of Orthopedics, Tochigi Medical Center Shimotsuga General Hospital, 5-32, Fujimicho, Tochigi-shi, Tochigi, 328-8505, Japan, nkm@jichi.ac.jp.
• European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society.Eur Spine J.2013 Aug 30. [Epub ahead of print]
• PURPOSE: Galactosialidosis is an autosomal recessive lysosomal storage disease caused by deficiency of both α-neuraminidase and β-galactosidase due to a defect of the protective protein/cathepsin A. Three clinical subtypes have been described, depending on the age of onset and severity of the symp
• PMID 23989749

• Galactosialidosis: review and analysis of CTSA gene mutations.

• Caciotti A, Catarzi S, Tonin R, Lugli L, Perez CR, Michelakakis H, Mavridou I, Donati MA, Guerrini R, d'Azzo A, Morrone A.SourceLaboratory for Molecular and Cell Biology, Paediatric Neurology Unit and Laboratories, Meyer Children's Hospital, Viale Pieraccini n, 24, Florence 50139, Italy.
• Orphanet journal of rare diseases.Orphanet J Rare Dis.2013 Aug 2;8:114. doi: 10.1186/1750-1172-8-114.
• BACKGROUND: Mutations in the CTSA gene, that encodes the protective protein/cathepsin A or PPCA, lead to the secondary deficiency of β-galactosidase (GLB1) and neuraminidase 1 (NEU1), causing the lysosomal storage disorder galactosialidosis (GS). Few clinical cases of GS have been reported in the l
• PMID 23915561

Japanese Journal

• Protective protein/cathepsin A down-regulates osteoclastogenesis by associating with and degrading NF-κB p50/p65

• MASUHARA Masaaki,SATO Takuya,HADA Naoto,HAKEDA Yoshiyuki
• Journal of bone and mineral metabolism 27(1), 46-56, 2009-01-30
• NAID 10025336150

• Elastogenesis in cultured dermal fibroblasts from patients withlysosomal β-galactosidase,protective protein/cathepsin A and neuraminidase-1 deficiencies

• Tatano Yutaka,Takeuchi Naohiro,Kuwahara Jun
• The journal of medical investigation : JMI 53(1/2), 103-112, 2006-02-00
• NAID 110004748823

• Expression of lysosomal protective protein/cathepsin A in a stably transformed human neuroblastoma cell line during bi-directional differentiation into neuronal and Schwannian cells

• Itoh K,Satoh Y,Kadota Y,Oheda Y,Kuwahara J,Shimmoto M,Sakuraba H
• NEUROCHEMISTRY INTERNATIONAL 44(6), 447-457, 2004-00-00
• NAID 120004292046

Related Links

• Lysosomal Protective Protein/Cathepsin A

Lysosomal Protective Protein/Cathepsin A - Free download as PDF File (.pdf), Text file (.txt) or read online for free. This report explores the role of lysosomal protective protein/cathepsin A and how defects in the gene encoding this ...

• Protective Protein/Cathepsin A Rescues N-glycosylation ...

Neuraminidase-1 (NEU1) catabolizes the hydrolysis of sialic acids from sialo-glycoconjugates. NEU1 depends on its interaction with the protective protein/cathepsin A (PPCA) for lysosomal compartmentalization and catalytic ...

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★リンクテーブル★

リンク元	「PPCA」
関連記事	「A」「cathepsin A」「Ad」「protective」

「PPCA」

　　[★]

同

protective protein/cathepsin A

「A」

　　[★]

• アデニン adenine
• アデノシン adenosine
• アラニン alanine

　 　 　

「cathepsin A」

　　[★]

カテプシンA

関

carboxypeptidase C、carboxypeptidase Y

「Ad」

　　[★]

• アデノウイルス

関

adenoviral、adenovirus

「protective」

　　[★]

• adj.

• 保護性の、保護的な