Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca(2+) stabilization
about
Functional amyloid formation within mammalian tissue.N-terminal region of gelsolin induces apoptosis of activated hepatic stellate cells by a caspase-dependent mechanismThe 8 and 5 kDa fragments of plasma gelsolin form amyloid fibrils by a nucleated polymerization mechanism, while the 68 kDa fragment is not amyloidogenicThe crystal structure of the C-terminus of adseverin reveals the actin-binding interface.Secretion of amyloidogenic gelsolin progressively compromises protein homeostasis leading to the intracellular aggregation of proteinsStructure of the N-terminal half of gelsolin bound to actin: roles in severing, apoptosis and FAFFurin at the cutting edge: from protein traffic to embryogenesis and diseaseCa2+ binding by domain 2 plays a critical role in the activation and stabilization of gelsolinAromatic Sulfonyl Fluorides Covalently Kinetically Stabilize Transthyretin to Prevent Amyloidogenesis while Affording a Fluorescent ConjugateAn ER-directed gelsolin nanobody targets the first step in amyloid formation in a gelsolin amyloidosis mouse modelSingle-molecule force spectroscopy reveals force-enhanced binding of calcium ions by gelsolinMetalloendoprotease cleavage triggers gelsolin amyloidogenesisCutis laxa in hereditary gelsolin amyloidosis.Severe ataxia with neuropathy in hereditary gelsolin amyloidosis: a case report.Therapeutic strategies for human amyloid diseases.Amyloid as a natural product.Formation of gelsolin amyloid fibrils in the rough endoplasmic reticulum of skeletal muscle in the gelsolin mouse model of inclusion body myositis: comparative analysis to human sporadic inclusion body myositisMolecular and cellular mechanisms of ectodomain sheddingThe Silver locus product Pmel17/gp100/Silv/ME20: controversial in name and in function.Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis.PMEL: a pigment cell-specific model for functional amyloid formationRegulatory role of the second gelsolin-like domain of Caenorhabditis elegans gelsolin-like protein 1 (GSNL-1) in its calcium-dependent conformation and actin-regulatory activities.Formation of Pmel17 amyloid is regulated by juxtamembrane metalloproteinase cleavage, and the resulting C-terminal fragment is a substrate for gamma-secretaseHacking the code of amyloid formation: the amyloid stretch hypothesis.Heparin accelerates gelsolin amyloidogenesis.Amyloidogenesis of natively unfolded proteins.Expression of BRI2 mRNA and protein in normal human brain and familial British dementia: its relevance to the pathogenesis of diseaseIntracellular substrate cleavage: a novel dimension in the biochemistry, biology and pathology of matrix metalloproteinases.Gelsolin amyloidosis: genetics, biochemistry, pathology and possible strategies for therapeutic intervention.Gelsolin: the tail of a molecular gymnast.Tryptophanyl substitutions in apomyoglobin determine protein aggregation and amyloid-like fibril formation at physiological pH.Molecular basis of a novel renal amyloidosis due to N184K gelsolin variant.Proneurotrophins require endocytosis and intracellular proteolysis to induce TrkA activation.Non-Invasive Imaging of Amyloid Deposits in a Mouse Model of AGel Using (99m)Tc-Modified Nanobodies and SPECT/CT.Gender differences in the clinical course of Finnish gelsolin amyloidosis.AAV9 delivered bispecific nanobody attenuates amyloid burden in the gelsolin amyloidosis mouse model.Discovery and characterization of a mammalian amyloid disaggregation activitySorting of furin in polarized epithelial and endothelial cells: expression beyond the Golgi apparatus.Chaperone nanobodies protect gelsolin against MT1-MMP degradation and alleviate amyloid burden in the gelsolin amyloidosis mouse model.Traffic jams II: an update of diseases of intracellular transport.
P2860
Q21092782-841E9FEA-5940-4B81-A8CC-9843A3765DD8Q24298188-F2D77AAC-C0C0-4540-BBDB-866022C7BF39Q24316253-AB3EDB05-05EB-4723-AA20-8AD9961050E5Q24322898-8DC3CA86-CE18-438D-A826-F02D935C362CQ24338969-B8DA9C9F-7A0F-47D8-A45C-F7E18013B91BQ24563146-CDED61C3-48B8-468A-AFB7-46583C072290Q24670432-1D2D049F-63F8-4878-8C39-963171BB6C7FQ27656937-00482C91-8D7A-4703-B7A1-79C7FCD99B4DQ27675997-4E88DBE6-85DE-4715-82D9-FD98C568E3D5Q27697593-132582FD-5C88-4C65-A8CC-FCF1161E52B3Q34086408-6A3E8BD2-430E-4DBF-88CC-6BFF4526B7E9Q34324885-C22F5ECC-456A-4536-8F95-77A55E72299DQ34396968-AB54D90A-152F-4E51-97CC-B88E09263DF6Q34622028-D2C91EE1-1EFE-4837-A88B-CD6699A16917Q34743288-1989D8B7-F6DB-44BD-92D2-45EF3FACFA36Q35125908-A452055F-2401-43DE-BAD2-5C0065A94005Q35704477-D0C65141-3262-4A55-A224-1005113AB65AQ36210626-17DA7031-E521-44AE-950D-D76333D3FE57Q36257261-4C80B929-8A1A-4647-9ECD-2E4DE884B769Q36323476-396777E0-419C-4ADE-9D15-E1BDC03506A3Q36788313-6F93A18B-17DF-46A5-AEFF-7726B75D9292Q36849236-D06A124B-F9B5-4D02-82F9-548AF12E20E1Q37068079-498C7E0A-EDA5-42C6-A4A4-8882D41B4FB3Q37079411-43527791-8042-4A08-9BA3-784C8C3A6A00Q37132636-D5CB87AC-211F-4B58-A635-F34611EB47A0Q37184755-7BCF001F-E76F-4093-9428-21A1FF122DB4Q37481580-B5AF69F8-2041-4D3B-8CD5-CBF348C14E5CQ37784801-B6660B6D-0DA0-4EE3-8ADB-448A1BC8A1A7Q37987587-7E7A9AAF-83E9-4874-BC8B-AFD31A5B3610Q38113172-63868AC7-5969-4970-97F7-ED684E507142Q38362738-5747A1DB-DA40-43A9-8859-C9DE7C567B85Q39385396-002B03C3-5BB1-4AEB-9043-FA60B7EB16B8Q39647271-0F20E508-EE17-4AEA-940B-BCFC2D379957Q39808570-F05D2BD5-A386-49F0-AEAE-94DB5D8305BFQ40067141-2B9CB6D2-44F7-45EC-91F0-25F402B673DDQ40277560-56CE7E21-440A-4B1E-8BF5-5376B16CB5A2Q40455022-8E8F677C-3C6C-4A16-9821-F6B9E6462F4EQ40564413-9F6443B3-777B-4DCB-9790-2215E84AF773Q40569183-35AB39D4-41FF-4D4A-BB96-37A86F5E569AQ40626944-5DC73E1F-E28A-44F3-8A89-F8ECB3DAF2D0
P2860
Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca(2+) stabilization
description
2001 nî lūn-bûn
@nan
2001 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca
@nl
Furin initiates gelsolin famil ...... defect in Ca(2+) stabilization
@ast
Furin initiates gelsolin famil ...... defect in Ca(2+) stabilization
@en
type
label
Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca
@nl
Furin initiates gelsolin famil ...... defect in Ca(2+) stabilization
@ast
Furin initiates gelsolin famil ...... defect in Ca(2+) stabilization
@en
prefLabel
Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca
@nl
Furin initiates gelsolin famil ...... defect in Ca(2+) stabilization
@ast
Furin initiates gelsolin famil ...... defect in Ca(2+) stabilization
@en
P2093
P2860
P356
P1433
P1476
Furin initiates gelsolin famil ...... defect in Ca(2+) stabilization
@en
P2093
J Matteson
R Phillips
P2860
P304
P356
10.1093/EMBOJ/20.22.6277
P407
P577
2001-11-01T00:00:00Z