Mutant tamm-horsfall glycoprotein accumulation in endoplasmic reticulum induces apoptosis reversed by colchicine and sodium 4-phenylbutyrate.
about
Uromodulin is expressed in renal primary cilia and UMOD mutations result in decreased ciliary uromodulin expressionA mouse model for inherited renal fibrosis associated with endoplasmic reticulum stress.Hereditary interstitial kidney diseaseChildhood course of renal insufficiency in a family with a uromodulin gene mutation.Molecular and cellular effects of Tamm-Horsfall protein mutations and their rescue by chemical chaperones.Uromodulin in kidney injury: an instigator, bystander, or protector?Mutant uromodulin expression leads to altered homeostasis of the endoplasmic reticulum and activates the unfolded protein response.Uromodulin upregulates TRPV5 by impairing caveolin-mediated endocytosis.Uromodulin mutations causing familial juvenile hyperuricaemic nephropathy lead to protein maturation defects and retention in the endoplasmic reticulumUromodulin: from physiology to rare and complex kidney disorders.Activation of the bumetanide-sensitive Na+,K+,2Cl- cotransporter (NKCC2) is facilitated by Tamm-Horsfall protein in a chloride-sensitive mannerFrom juvenile hyperuricaemia to dysfunctional uromodulin: an ongoing metamorphosis.No amelioration of uromodulin maturation and trafficking defect by sodium 4-phenylbutyrate in vivo: studies in mouse models of uromodulin-associated kidney disease.A novel homozygous UMOD mutation reveals gene dosage effects on uromodulin processing and urinary excretion.Novel missense mutation of uromodulin in mice causes renal dysfunction with alterations in urea handling, energy, and bone metabolism.High dietary methionine plus cholesterol stimulates early atherosclerosis and late fibrous cap development which is associated with a decrease in GRP78 positive plaque cells.A transgenic mouse model for uromodulin-associated kidney diseases shows specific tubulo-interstitial damage, urinary concentrating defect and renal failure.Endoplasmic reticulum stress in the pathogenesis of fibrotic disease.Biallelic Expression of Mucin-1 in Autosomal Dominant Tubulointerstitial Kidney Disease: Implications for Nongenetic Disease Recognition
P2860
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P2860
Mutant tamm-horsfall glycoprotein accumulation in endoplasmic reticulum induces apoptosis reversed by colchicine and sodium 4-phenylbutyrate.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年学术文章
@wuu
2005年学术文章
@zh
2005年学术文章
@zh-cn
2005年学术文章
@zh-hans
2005年学术文章
@zh-my
2005年学术文章
@zh-sg
2005年學術文章
@yue
2005年學術文章
@zh-hant
name
Mutant tamm-horsfall glycoprot ...... e and sodium 4-phenylbutyrate.
@en
Mutant tamm-horsfall glycoprot ...... e and sodium 4-phenylbutyrate.
@nl
type
label
Mutant tamm-horsfall glycoprot ...... e and sodium 4-phenylbutyrate.
@en
Mutant tamm-horsfall glycoprot ...... e and sodium 4-phenylbutyrate.
@nl
prefLabel
Mutant tamm-horsfall glycoprot ...... e and sodium 4-phenylbutyrate.
@en
Mutant tamm-horsfall glycoprot ...... e and sodium 4-phenylbutyrate.
@nl
P2093
P356
P1476
Mutant tamm-horsfall glycoprot ...... e and sodium 4-phenylbutyrate.
@en
P2093
Anthony J Bleyer
In Sun Song
Ok Hee Ryu
Sun Jin Choi
Sung Won Choi
Thomas C Hart
P304
P356
10.1681/ASN.2005050461
P577
2005-08-31T00:00:00Z