Rab geranylgeranyl transferase alpha mutation in the gunmetal mouse reduces Rab prenylation and platelet synthesis
about
Protein prenyltransferasesBLOC-1, a novel complex containing the pallidin and muted proteins involved in the biogenesis of melanosomes and platelet-dense granulesThe Hermansky-Pudlak syndrome 1 (HPS1) and HPS4 proteins are components of two complexes, BLOC-3 and BLOC-4, involved in the biogenesis of lysosome-related organellesBLOC-3, a protein complex containing the Hermansky-Pudlak syndrome gene products HPS1 and HPS4BLOC-1 is required for cargo-specific sorting from vacuolar early endosomes toward lysosome-related organellesThe Rab27a/granuphilin complex regulates the exocytosis of insulin-containing dense-core granulesFunctional redundancy of Rab27 proteins and the pathogenesis of Griscelli syndromeThe gene for the muted (mu) mouse, a model for Hermansky-Pudlak syndrome, defines a novel protein which regulates vesicle traffickingHermansky-Pudlak syndrome type 7 (HPS-7) results from mutant dysbindin, a member of the biogenesis of lysosome-related organelles complex 1 (BLOC-1)Melanosomes--dark organelles enlighten endosomal membrane transportMelanocyte-specific proteins are aberrantly trafficked in melanocytes of Hermansky-Pudlak syndrome-type 3Rab and Arf proteins in genetic diseasesRegulation of osteoclasts by membrane-derived lipid mediatorsMolecular evolution of the Rab-escort-protein/guanine-nucleotide-dissociation-inhibitor superfamilyAssembly of the biogenesis of lysosome-related organelles complex-3 (BLOC-3) and its interaction with Rab9BLOC-3 mutated in Hermansky-Pudlak syndrome is a Rab32/38 guanine nucleotide exchange factorRab27a regulates the peripheral distribution of melanosomes in melanocytesRab38 targets to lamellar bodies and normalizes their sizes in lung alveolar type II epithelial cells.Expression analysis of primary mouse megakaryocyte differentiation and its application in identifying stage-specific molecular markers and a novel transcriptional target of NF-E2.Mechanism of platelet factor 4 (PF4) deficiency with RUNX1 haplodeficiency: RUNX1 is a transcriptional regulator of PF4.Inherited platelet disorders: a clinical approach to diagnosis and management.Evidence for defective Rab GTPase-dependent cargo traffic in immune disorders.Analysis of the lysosomal storage disease Chediak-Higashi syndrome.Hermansky-Pudlak syndrome and related disorders of organelle formation.Megakaryocytes and beyond: the birth of platelets.Multiple factors contribute to inefficient prenylation of Rab27a in Rab prenylation diseases.Independent degeneration of photoreceptors and retinal pigment epithelium in conditional knockout mouse models of choroideremia.Normal and abnormal secretion by haemopoietic cells.Rab23, a negative regulator of hedgehog signaling, localizes to the plasma membrane and the endocytic pathway.Prenylation of Rab GTPases: molecular mechanisms and involvement in genetic disease.Hermansky-Pudlak syndrome: a disease of protein trafficking and organelle function.The dark side of lysosome-related organelles: specialization of the endocytic pathway for melanosome biogenesis.The secretory synapse: the secrets of a serial killer.Hermansky-Pudlak syndrome: vesicle formation from yeast to man.Impaired prenylation of Rab GTPases in the gunmetal mouse causes defects in bone cell function.Mevalonate Pathway Regulates Cell Size Homeostasis and Proteostasis through AutophagyRab escort protein 1 (REP1) in intracellular traffic: a functional and pathophysiological overview.Mechanisms of protein delivery to melanosomes in pigment cells.The cell biology of Hermansky-Pudlak syndrome: recent advances.Thematic review series: lipid posttranslational modifications. geranylgeranylation of Rab GTPases.
P2860
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P2860
Rab geranylgeranyl transferase alpha mutation in the gunmetal mouse reduces Rab prenylation and platelet synthesis
description
2000 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
article publié dans les Procee ...... f the United States of America
@fr
artículu científicu espublizáu en 2000
@ast
im April 2000 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2000/04/11)
@sk
vědecký článek publikovaný v roce 2000
@cs
wetenschappelijk artikel (gepubliceerd op 2000/04/11)
@nl
наукова стаття, опублікована у квітні 2000
@uk
name
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@ast
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@en
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@nl
type
label
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@ast
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@en
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@nl
prefLabel
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@ast
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@en
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@nl
P2093
P2860
P3181
P356
P1476
Rab geranylgeranyl transferase ...... ylation and platelet synthesis
@en
P2093
E. B. McMurtrie
E. H. Mules
E. K. Novak
J. C. Detter
M. C. Seabra
M. R. Wallace
R. T. Swank
S. F. Kingsmore
V. S. Mishra
P2860
P304
P3181
P356
10.1073/PNAS.080517697
P407
P577
2000-04-11T00:00:00Z