Defective pro alpha 2(I) collagen synthesis in a recessive mutation in mice: a model of human osteogenesis imperfecta. - Wikidata - awgldk - TriplyDB

Defective pro alpha 2(I) collagen synthesis in a recessive mutation in mice: a model of human osteogenesis imperfecta.

Defective pro alpha 2(I) collagen synthesis in a recessive mutation in mice: a model of human osteogenesis imperfecta.

http://www.wikidata.org/entity/Q36125438

about

ER stress-mediated apoptosis in a new mouse model of osteogenesis imperfecta Use of the Cre/lox recombination system to develop a non-lethal knock-in murine model for osteogenesis imperfecta with an alpha1(I) G349C substitution. Variability in phenotype in BrtlIV mice The NH2-terminal propeptide of type I procollagen acts intracellularly to modulate cell function Relation of PvuII site polymorphism in the COL1A2 gene to the risk of fractures in prepubertal Finnish girls.Segregation of type I collagen homo- and heterotrimers in fibrils Immature osteoblast lineage cells increase osteoclastogenesis in osteogenesis imperfecta murine.Muscle abnormalities in osteogenesis imperfecta.Potential implications of cell therapy for osteogenesis imperfecta.Carcinomas contain a matrix metalloproteinase-resistant isoform of type I collagen exerting selective support to invasion Defective splicing of mRNA from one COL1A1 allele of type I collagen in nondeforming (type I) osteogenesis imperfecta.Rare autosomal recessive cardiac valvular form of Ehlers-Danlos syndrome results from mutations in the COL1A2 gene that activate the nonsense-mediated RNA decay pathway.Amelioration of a mouse model of osteogenesis imperfecta with hematopoietic stem cell transplantation: microcomputed tomography studies.Targeting the LRP5 pathway improves bone properties in a mouse model of osteogenesis imperfecta.Increased resorptive activity and accompanying morphological alterations in osteoclasts derived from the oim/oim mouse model of osteogenesis imperfecta.Abnormal mineral-matrix interactions are a significant contributor to fragility in oim/oim bone Skeletal muscle weakness in osteogenesis imperfecta mice Extracellular microfibrils control osteoblast-supported osteoclastogenesis by restricting TGF{beta} stimulation of RANKL production RANKL inhibition improves bone properties in a mouse model of osteogenesis imperfecta.Collagen mutation causes changes of the microdamage morphology in bone of an OI mouse model Prenatal transplantation of mesenchymal stem cells to treat osteogenesis imperfecta.Myostatin deficiency partially rescues the bone phenotype of osteogenesis imperfecta model mice.Decreasing maternal myostatin programs adult offspring bone strength in a mouse model of osteogenesis imperfecta Second-harmonic generation circular dichroism studies of osteogenesis imperfecta Variable bone fragility associated with an Amish COL1A2 variant and a knock-in mouse model.Deficient degradation of homotrimeric type I collagen, α1(I)3 glomerulopathy in oim mice.Gene therapy approaches for osteogenesis imperfecta.Structural and mechanical differences between collagen homo- and heterotrimers: relevance for the molecular origin of brittle bone disease.How tough is brittle bone? Investigating osteogenesis imperfecta in mouse bone.Are Changes in Composition in Response to Treatment of a Mouse Model of Osteogenesis Imperfecta Sex-dependent?Altered trabecular bone structure and delayed cartilage degeneration in the knees of collagen VI null mice An investigation of the mineral in ductile and brittle cortical mouse bone.The role of SH3BP2 in the pathophysiology of cherubism.OIM and related animal models of osteogenesis imperfecta.A COL1A1 Sp1 binding site polymorphism predisposes to osteoporotic fracture by affecting bone density and quality.New mouse models for metabolic bone diseases generated by genome-wide ENU mutagenesis.Ultra-structural defects cause low bone matrix stiffness despite high mineralization in osteogenesis imperfecta mice.Structure-mechanics relationships of collagen fibrils in the osteogenesis imperfecta mouse model.Transplanted bone marrow mononuclear cells and MSCs impart clinical benefit to children with osteogenesis imperfecta through different mechanisms.Bulbous epiphysis and popcorn calcification as related to growth plate differentiation in osteogenesis imperfecta Increased Young's modulus and hardness of Col1a2oim dentin.

P2860

Q21563327-ABD396BE-4195-4167-9E2C-AF5B77483E66 Q28507737-B92A9C97-099A-4815-831C-CC353F20160F Q28510648-6462DB97-E534-47D4-881B-E1B727D2A9FF Q33187450-8FA128C7-8811-441D-89CC-34E942F2C4C7 Q33730397-D27467BA-9A12-4FD6-A03F-B79914976867 Q33816415-F597F8EF-8574-471E-8C9A-EE953E55C7FD Q33856898-939864C7-BC35-4B60-A810-F5D80D1AEC57 Q33863266-C2ACEC5F-6604-4CB8-89C5-070612F7DA06 Q33892014-5A9E1438-3C4B-486A-9E3F-B0437C56E405 Q33905897-47F6572D-1931-470B-9767-9B626E8D3D23 Q33909884-AAEC29A1-9E07-47AA-A2FB-4189FEF80A7A Q33958410-D5EE6FC9-DAB7-450D-B51F-37FCFE1EDF60 Q34036439-BC415E7F-B48E-4E17-9192-96FEE3966BE6 Q34148428-AA830BB1-FCFE-4CEB-9D2B-6223A1149917 Q34153396-5033373B-EF23-4C99-B5F8-FD33B21C4917 Q34188512-C9276648-34F9-435A-846D-DCDBB3F6B904 Q34232152-4AF750A9-ACFD-4F4A-9096-DF812E3E30A2 Q34234347-84927503-5595-4BAB-B638-FD51C1B4F756 Q34268022-90667946-7416-4DCA-BF05-58BE40D04CF6 Q34312131-F7B1A88C-7217-4EF1-A522-8642AD169173 Q34485335-AE409F1B-43E5-4EBB-B26A-5C14413465AE Q34544274-100F9126-0AED-4F3B-B267-CE10AAF6E383 Q35112256-D0F611BA-DC2F-4762-B2AC-23D01B06243C Q35156234-2862CB96-265A-49D3-B4F3-27F0E178CD94 Q35489554-354F8EB5-8113-466C-806F-E78E573973C2 Q35629569-8825BADF-6095-4134-9E6B-F0E2E5977BBF Q35743248-31FF881D-A7B2-46C3-A6FB-C7D9897A7CBC Q35771774-41ED2642-5591-48C9-AA53-B7B5FF2FB492 Q35807492-93077E55-D7AE-470C-85B9-85B6D4508CEC Q35842479-2A798E4C-DB77-44B4-BF97-732AA6C13B75 Q35866985-D019E03F-14A3-474D-9DF4-6B01108C1E39 Q35987370-E3C1FCF2-CE74-49F3-8B27-434AA9CF2C02 Q35990573-60A32DCF-7C6F-49A9-BAB1-F47A5E1CE892 Q36016930-3A3CE6FD-3198-441D-88E4-0C7D648DB838 Q36106531-D6801C17-AE29-4B0C-99CA-BD6CFEC2F198 Q36125953-B08F6ED9-9A2A-48F2-8EBA-62D68F888BA9 Q36187857-A082F583-00ED-40A5-B6E4-9DD23163D242 Q36206449-95E3BF3B-1ED4-41C4-AD22-0793090EA6A7 Q36223620-A7BF6B6F-9E8A-4B41-9D48-BA7A93E9C582 Q36464958-C24E183F-1CFF-444C-93FB-267BCB8F5DD3

P2860

Defective pro alpha 2(I) collagen synthesis in a recessive mutation in mice: a model of human osteogenesis imperfecta.

http://www.wikidata.org/entity/Q36125438

description

1993 nî lūn-bûn

@nan

1993年の論文

@ja

1993年論文

@yue

1993年論文

@zh-hant

1993年論文

@zh-hk

1993年論文

@zh-mo

1993年論文

@zh-tw

1993年论文

@wuu

1993年论文

@zh

1993年论文

@zh-cn

name

Defective pro alpha 2(I) colla ...... human osteogenesis imperfecta.

@ast

Defective pro alpha 2(I) colla ...... human osteogenesis imperfecta.

@en

type

label

Defective pro alpha 2(I) colla ...... human osteogenesis imperfecta.

@ast

Defective pro alpha 2(I) colla ...... human osteogenesis imperfecta.

@en

prefLabel

Defective pro alpha 2(I) colla ...... human osteogenesis imperfecta.

@ast

Defective pro alpha 2(I) colla ...... human osteogenesis imperfecta.

@en

P1433

Q36125438-A0E24FC9-7000-41D0-888E-AADE0BC8B554

P1476

Q36125438-70811AA0-F2C7-490F-A182-11E8FE96205C

P2093

Q36125438-0A780DF9-C563-4CD3-883B-7C4CF3E36B0E

Q36125438-0B6E5374-375D-490C-B8F7-10F7795A6F7C

Q36125438-1107895E-1648-4475-BD14-ACFF7B18201D

Q36125438-307EF5C5-B598-456E-B407-7693E69D4B10

Q36125438-93517B66-0B37-4692-A5E3-5D1C0DA5A367

Q36125438-B4FDBD7D-D62B-455F-A926-A6ABE3EF23B3

Q36125438-D616FFFC-569F-406C-A30D-9EE988CA0999

Q36125438-E8EEC817-D36D-4F7D-97F8-1C9C972BFCEA

Q36125438-EDEDC06D-372D-46E7-B8F6-2CDC8A2F2BFC

P2860

Q36125438-02C4F3F9-A9C7-4095-9F4A-D6F36BE49BD2

Q36125438-0A67DEDA-620E-4449-AFD2-B31E7263AEDC

Q36125438-126B3A2E-16B7-4842-B76C-005403C9213F

Q36125438-13D9E342-98AE-4A30-81E3-04E96153272B

Q36125438-20564BC1-9F24-4F47-BCA6-FD9476F1143E

Q36125438-21BDE615-730E-4EF9-BF25-FE9F87B92C9C

Q36125438-30661F45-49CC-4FAB-B1A6-B2B5D69335F5

Q36125438-36A66598-7C99-43E7-ADB4-EDA2A4F23F87

Q36125438-374E957D-8DD0-4CC9-9C1B-DBD1D77DCE2C

Q36125438-3CB27208-F288-4640-A7E6-595AA6FABC67

P304

Q36125438-C73C02A8-2FD4-4A46-AFBF-31E2A4A88F43

P31

Q36125438-E47AA151-8FA5-458F-AF0B-B62EFF8D2B94

P356

Q36125438-3A1B9346-43E8-4EA9-90C1-BF78A48BE51F

P407

Q36125438-5284442C-3D63-4A73-8018-3F786419300A

P433

Q36125438-7F6B5C61-7E4B-4595-812B-AC4E745615B8

P478

Q36125438-D3622DDD-E3D2-4739-B164-1FD972D29D94

P577

Q36125438-4F7667D7-ABB9-4F59-88A1-65BB94B324C4

P698

Q36125438-D8173862-9B1E-45B8-BC66-AA7239B50A0F

P921

Q36125438-2E2ED1C5-7450-4FBD-A70C-A81E8E69F7D4

P932

Q36125438-D3920AD5-B4BB-4377-ACFF-BFFA52E9AD2A

P356

P1433

Proceedings of the National Academy of Sciences of the United States of America

P1476

Defective pro alpha 2(I) colla ...... human osteogenesis imperfecta.

@en

P2093

A R Shuldiner

http://www.w3.org/2001/XMLSchema#string

D J McBride

http://www.w3.org/2001/XMLSchema#string

D W Rowe

http://www.w3.org/2001/XMLSchema#string

H O Sweet

http://www.w3.org/2001/XMLSchema#string

J R Shapiro

http://www.w3.org/2001/XMLSchema#string

M T Davisson

http://www.w3.org/2001/XMLSchema#string

R J Wenstrup

http://www.w3.org/2001/XMLSchema#string

S C Marks

http://www.w3.org/2001/XMLSchema#string

S D Chipman

http://www.w3.org/2001/XMLSchema#string

P2860

Transgenic mouse model of the mild dominant form of osteogenesis imperfecta

Rapid production of full-length cDNAs from rare transcripts: amplification using a single gene-specific oligonucleotide primer

Construction of a full-length murine pro alpha 2(I) collagen cDNA by the polymerase chain reaction.

Recurrence risks and prognosis in severe sporadic osteogenesis imperfecta.

Introduction of the human pro alpha 1(I) collagen gene into pro alpha 1(I)-deficient Mov-13 mouse cells leads to formation of functional mouse-human hybrid type I collagen

Insertion of retrovirus into the first intron of alpha 1(I) collagen gene to embryonic lethal mutation in mice.

Osteonectin, bone proteoglycan, and phosphophoryn defects in a form of bovine osteogenesis imperfecta.

Nuclease S1 mapping of a homozygous mutation in the carboxyl-propeptide-coding region of the pro alpha 2(I) collagen gene in a patient with osteogenesis imperfecta.

Rescue of type I collagen-deficient phenotype by retroviral-vector-mediated transfer of human pro alpha 1(I) collagen gene into Mov-13 cells

Retrovirus-induced de novo methylation of flanking host sequences correlates with gene inactivity.

P304

1701-1705

http://www.w3.org/2001/XMLSchema#string

P31

scholarly article

P356

10.1073/PNAS.90.5.1701

http://www.w3.org/2001/XMLSchema#string

P407

P433

5

http://www.w3.org/2001/XMLSchema#string

P478

90

http://www.w3.org/2001/XMLSchema#string

P577

1993-03-01T00:00:00Z

http://www.w3.org/2001/XMLSchema#dateTime

P698

8446583

http://www.w3.org/2001/XMLSchema#string

P921

osteogenesis imperfecta

P932

45947

http://www.w3.org/2001/XMLSchema#string