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TranscriptomicsTranscription profiling of mouse deficient for Fibrillin-1
Different fibrillin-1 mutant mice
ORGANISM(S): Mus musculus 
2008-06-11 | E-GEOD-1852 | biostudies-arrayexpress
ProteomicsTissue Microfibril Comparison - Structural and compositional diversity of fibrillin microfibrils in human tissues
Fibrillin microfibrils are large supramolecular ECM assemblies which play different functional roles in different tissues. We aim to compare the ultrastructure and biological composition of fibrillin microfibrils purified from human eye, skin and cultured dermal fibroblasts. This will allow us to un...
ORGANISM(S): Homo sapiens (Human) 
2018-02-16 | PXD008450 | Pride
Human Microfibril Afm Fibrillin Collagen Lc-ms/ms 
ProteomicsProteolysis of fibrillin-2 microfibrils is essential for normal skeletal development
Analysis of mouse Adamts6 and Adamts10 mutant embryos, lacking these homologous secreted metalloproteases individually and in combination, along with in vitro analysis of microfibrils, measurement of ADAMTS6-fibrillin affinities and N-terminomics determination of ADAMTS6-cleaved sites, demonstrates ...
ORGANISM(S): Homo sapiens (Human) 
2022-05-20 | PXD027096 | Pride
Fibrillin Fibronectin Hek293t Adamts6 Extracellular matrix 
TranscriptomicsFibrillin-1 deficient mice
Different fibrillin-1 mutant mice Keywords: other
ORGANISM(S): Mus musculus 
2004-10-20 | GSE1852 | GEO
ProteomicsLoss of POGLUT2 and 3 eliminates O-glucose at predicted sites but does not alter other modifications on EGFs
Fibrillins play a critical role in tissue/organ development and cardiopulmonary function. Here we showed that O-glucosylation of the fibrillins’ epidermal growth factor-like repeats by protein O-glucosyltransferase 2 (POGLUT2) and POGLUT3 was indispensable for in vivo function of mouse fibrillins. L...
ORGANISM(S): Mus Musculus 
2024-06-10 | PXD043827 | panorama
ProteomicsSecretome analysis of mouse fibroblast
Fibrillin-1, an extracellular matrix (ECM) protein encoded by FBN1 gene, serves as the microfibril scaffold crucial for elastic fiber formation and homeostasis in pliable tissue such as the skin. Besides causing Marfan syndrome, some mutations in FBN1 result in scleroderma, marked by hardened and th...
ORGANISM(S): Mus Musculus (mouse) 
2025-04-11 | PXD052580 | JPOST Repository
GenomicsFibrillin-Related Proteins Control Calcium Homeostasis in Dystrophic Muscle Across Species
Fibrillin-Related Proteins Control Calcium Homeostasis in Dystrophic Muscle Across Species
PRJNA1275763 | ENA
GenomicsSystematic disruption of zebrafish fibrillin genes identifies a translational zebrafish model for Marfan syndrome
Systematic disruption of zebrafish fibrillin genes identifies a translational zebrafish model for Marfan syndrome
PRJNA1280187 | ENA
TranscriptomicsTranscription profiling of lungs of wild type and Mg-delta strain of fibrillin-1deficient mice to identify pathways that are critical for normal alveolar septation
The Mg-delta strain of fibrillin-1 deficient mice display an early defect in alveolar septation. This study attempts to identify expression patterns in the mutant mouse lung compared to their wild-type littermates that suggest pathways that are critical for normal septation. This experiment focuse...
ORGANISM(S): Mus musculus 
2007-11-06 | E-GEOD-479 | biostudies-arrayexpress
UnknownMaterial and mechanical properties of bones deficient for fibrillin-1 or fibrillin-2 microfibrils.
Not available
S-EPMC3097426 | biostudies-literature
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