The immunophenotypic and immunogenotypic B-cell differentiation arrest in bone marrow of RAG-deficient SCID patients corresponds to residual recombination activities of mutated RAG proteins.
about
The plant homeodomain finger of RAG2 recognizes histone H3 methylated at both lysine-4 and arginine-2Human RAG mutations: biochemistry and clinical implicationsAnalysis of mutations from SCID and Omenn syndrome patients reveals the central role of the Rag2 PHD domain in regulating V(D)J recombination.PHD fingers: epigenetic effectors and potential drug targetsHomeostatic expansion of autoreactive immunoglobulin-secreting cells in the Rag2 mouse model of Omenn syndromeA variant of SCID with specific immune responses and predominance of gamma delta T cells.A novel immunodeficiency associated with hypomorphic RAG1 mutations and CMV infection.A new type of radiosensitive T-B-NK+ severe combined immunodeficiency caused by a LIG4 mutation.DNA double-strand break signaling and human disorders.Loss of p19Arf in a Rag1(-/-) B-cell precursor population initiates acute B-lymphoblastic leukemia.Hypogammaglobulinemia in BLT humanized mice--an animal model of primary antibody deficiency.A hypomorphic R229Q Rag2 mouse mutant recapitulates human Omenn syndrome.B-cell development and functions and therapeutic options in adenosine deaminase-deficient patients.A plant homeodomain in RAG-2 that binds Hypermethylated lysine 4 of histone H3 is necessary for efficient antigen-receptor-gene rearrangement.A DNA-PKcs mutation in a radiosensitive T-B- SCID patient inhibits Artemis activation and nonhomologous end-joining.Newborn screening for severe combined immunodeficiency and T-cell lymphopenia in California: results of the first 2 yearsPHD fingers in human diseases: disorders arising from misinterpreting epigenetic marks.Temporal and spatial regulation of V(D)J recombination: interactions of extrinsic factors with the RAG complex.Educational paper. The expanding clinical and immunological spectrum of severe combined immunodeficiency.Wiskott-Aldrich Syndrome protein deficiency perturbs the homeostasis of B-cell compartment in humans.XLF deficiency results in reduced N-nucleotide addition during V(D)J recombination.Decreased IL7Rα and TdT expression underlie the skewed immunoglobulin repertoire of human B-cell precursors from fetal originDNA recombination defects in Kuwait: Clinical, immunologic and genetic profile.
P2860
Q27649107-2624086F-D8A4-4830-8BB3-4A53584CCCDBQ28274274-7BC158E2-DF71-4237-B14B-2F0CD52B268EQ33755407-8C7966CC-328F-4803-9BCA-C5A3CCAEA03EQ33819263-FCA5E17A-447F-4CCC-B698-8CAB51B4B019Q33979830-C975D3C9-906D-406B-8961-AB274BC3070CQ34063158-AD4FED13-A429-4DEE-9F9D-30F7895F1386Q34097544-77517809-2634-4125-9398-3A4D294DBF0EQ34211015-1A7562D1-6AAA-4E9A-9953-C712A0367C76Q34360500-246824F1-D18D-4A92-AA93-CEB9244579A6Q35125898-B295C2D5-F0A9-4871-8BF1-EFD330F6FB02Q35291397-330EDF43-CBB2-4F96-88FC-0FB5AE6E19EBQ35762099-8C0A17CD-C5A5-4149-954E-F63FB09C0CC0Q35810860-947A66B1-4599-4512-AD51-27223D5180D1Q37013832-0F536164-3117-474D-AD5D-D579429E6D9EQ37036037-74ACE8BF-727F-4DBC-96B3-1D3A1C77B121Q37137721-3118BDE2-D79F-4C08-B67F-4AC53C589BABQ37235746-7F377D29-3544-4203-8FFA-15CBFCBC47CEQ37592212-065CFF7F-EA3A-4A67-A885-5CED17965C50Q37863509-49A7A5EB-AF2C-4454-8A37-C19B35C10C59Q39306145-8BF89D9B-A518-4916-B8B6-3BC499A102D3Q39696233-6FBF9934-C41D-4F96-B387-211554EB9A24Q41164082-3A5D4433-80D1-4BDB-9BE2-CC1D050D630DQ50099202-9CCEE016-4DE1-46B1-9EE1-95AEE4DAD855
P2860
The immunophenotypic and immunogenotypic B-cell differentiation arrest in bone marrow of RAG-deficient SCID patients corresponds to residual recombination activities of mutated RAG proteins.
description
2002 nî lūn-bûn
@nan
2002年の論文
@ja
2002年学术文章
@wuu
2002年学术文章
@zh
2002年学术文章
@zh-cn
2002年学术文章
@zh-hans
2002年学术文章
@zh-my
2002年学术文章
@zh-sg
2002年學術文章
@yue
2002年學術文章
@zh-hant
name
The immunophenotypic and immun ...... ities of mutated RAG proteins.
@en
The immunophenotypic and immun ...... ities of mutated RAG proteins.
@nl
type
label
The immunophenotypic and immun ...... ities of mutated RAG proteins.
@en
The immunophenotypic and immun ...... ities of mutated RAG proteins.
@nl
prefLabel
The immunophenotypic and immun ...... ities of mutated RAG proteins.
@en
The immunophenotypic and immun ...... ities of mutated RAG proteins.
@nl
P2093
P1433
P1476
The immunophenotypic and immun ...... vities of mutated RAG proteins
@en
P2093
Anton W Langerak
Dik C van Gent
Ewa Bernatowska
Jaak M J J Vossen
Nicole S Verkaik
Ronald de Groot
Sandra de Bruin-Versteeg
P304
P407
P577
2002-09-01T00:00:00Z