RAG2 - recombination activating 2 Gene

Homo sapiens

Also known as RAG-2

Gene ID: 5897 | Gene type: protein coding

About RAG2

Cytogenetic location: 11p12 Genomic coordinates (GRCh38): 11:36,590,996-36,598,236 (from NCBI)

This gene has 8 transcripts (splice variants), 188 orthologues and is associated with 7 phenotypes. Restricted expression toward thyroid (RPKM 16.6).

Summary

This gene encodes a protein that is involved in the initiation of V(D)J recombination during B and T cell development. This protein forms a complex with the product of the adjacent recombination activating gene 1, and this complex can form double-strand breaks by cleaving DNA at conserved recombination signal sequences. The recombination activating gene 1 component is thought to contain most of the catalytic activity, while the N-terminal of the recombination activating gene 2 component is thought to form a six-bladed propeller in the active core that serves as a binding scaffold for the tight association of the complex with DNA. A C-terminal plant homeodomain finger-like motif in this protein is necessary for interactions with chromatin components, specifically with histone H3 that is trimethylated at lysine 4. Mutations in this gene cause Omenn syndrome, a form of severe combined immunodeficiency associated with autoimmune-like symptoms. [provided by RefSeq, Jul 2008]

RAG2 Products(3)

mRNA	Protein	Name
NM_000536.4	NP_000527.2	V(D)J recombination-activating protein 2
NM_001243785.2	NP_001230714.1	V(D)J recombination-activating protein 2
NM_001243786.2	NP_001230715.1	V(D)J recombination-activating protein 2

RAG2 Protein Structure

RAG2

RAG2_PHD

0
100
200
300
400
527 a.a.

Protein Preferred Names

Protein Names

V(D)J recombination-activating protein 2

recombination activating gene 2

Related Diseases

Diseases

Alias

Omenn Syndrome

Histiocytic Medullary Reticulosis	Severe Combined Immunodeficiency With Hypereosinophilia
Combined Immunodeficiency With Hypereosinophilia	Reticuloendotheliosis, Familial, With Eosinophilia
Reticuloendotheliosis Familial With Eosinophilia	Familial Reticuloendotheliosis
Omenn'S Syndrome	OS
Malignant Histiocytosis

Combined Cellular And Humoral Immune Defects With Granulomas

Combined Immunodeficiency With Skin Granulomas	CCHIDG
Cid Due To Rag 1/2 Deficiency	Combined Immunodeficiency Due To Rag 1/2 Deficiency
Combined Immunodeficiency With Granulomatosis	CHIDG
Immune Defects, Combined Cellular And Humoral With Granulomas

Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-Negative, B Cell-Positive, Nk Cell-Negative

Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-Negative, B Cell-Negative, Nk Cell-Positive	Severe Combined Immunodeficiency, B Cell-Negative
T-B+ Severe Combined Immunodeficiency Due To Jak3 Deficiency	Severe Combined Immunodeficiency Due To Complete Rag1/2 Deficiency
Scid Due To Complete Rag1/2 Deficiency	T B NK SCID
Scid, T Cell-Negative, B Cell-Positive, Nk Cell-Negative	Scid, T Cell-Negative, B Cell-Negative, Nk Cell-Positive
Scid, Autosomal Recessive, T-Negative/B-Positive Type	Autosomal Recessive T Cell-Negative, B-Cell Negative, Nk Cell-Positive Scid
Scid Due To Complete Rag1-2 Deficiency	Severe Combined Immunodeficiency Due To Complete Rag1-2 Deficiency
Scid, Ar, T-Cell Negative, B-Cell Negative, Nk Cell-Positive	Severe Immunodeficiency, Autosomal Recessive, T-Cell Negative, B-Cell Negative, Nk Cell-Positive
Jak3-Deficient Severe Combined Immunodeficiency	Autosomal Recessive T Cell-Negative, B Cell-Positive, Nk Cell-Negative Severe Combined Immunodeficiency
Autosomal Recessive T-B+Nk- Scid	Jak3 Scid
T Cell-Negative, B Cell-Positive, Nk Cell-Negative Scid	T-B+ Scid Due To Jak3 Deficiency
Severe Combined Immunodeficiency Autosomal Recessive T-Cell-Negative/B-Cell-Negative/Nk-Cell-Positive	Scid T Cell-Negative B Cell-Negative Nk Cell-Positive
Severe Combined Immunodeficiency Autosomal Recessive T Cell-Negative/B Cell-Negative/Nk Cell-Positive	Severe Combined Immunodeficiency Autosomal Recessive T-Cell Negative/B-Cell Negative/Nk-Cell Positive
Severe Combined Immunodeficiency Autosomal Recessive T-Cell-Negative/B-Cell-Positive/Nk-Cell-Negative	Severe Combined Immunodeficiency Autosomal Recessive T Cell-Negative/B Cell-Positive/Nk Cell-Negative
Severe Combined Immunodeficiency Autosomal Recessive T-Cell Negative/B-Cell Positive/Nk-Cell Negative	Immunodeficiency, Severe Combined, Autosomal Recessive, T Cell-Negative, B Cell-Positive, Nk Cell-Negative
Immunodeficiency, Severe Combined, Autosomal Recessive, T-Cell Negative, B-Cell Negative, Nk Cell-Positive

Immune Deficiency Disease

Immunodeficiency	Primary Immunodeficiency
Primary Immunodeficiency Disease	Immunologic Deficiency Syndromes
Hypoimmunity	Immune Deficiency Disorder
Immunodeficiency Syndrome	Immune Disorder
Primary Immune Deficiency Disorder	Immune System Diseases
Human Immunodeficiency Virus Infection	Hiv - [Human Immunodeficiency Virus Infection]
Hiv Positive Nos	Hiv Disease
Acquired Immune Deficiency Syndrome-Related Complex	Aids-Like Syndrome
Aids-Related Complex Nos	Arc - [Aids-Related Complex]
Immunodeficiency Due To Human Immunodeficiency Virus Infection	Unspecified Human Immunodeficiency Virus Disease
Hiv Disease Nos	Human Immunodeficiency Virus Positive Nos
Hiv Nos	Deficiency Of Complement Initial Pathway
Deficiency Of Complement Terminal Pathway	Cfdd - [Complement Factor D Deficiency]
Immunodeficiency With Nk-Cell - [Natural-Killer Cell] Deficiency	Nonfamilial Hypogammaglobulinaemia
Common Variable Immune Deficiency	Nonfamilial Agammaglobulinaemia
Common Variable Agammaglobulinaemia	Agammaglobulinaemia Nos
Agammaglobulinaemia Antibody Deficiency Syndrome	Hypogammaglobulinaemia Antibody Deficiency Syndrome
Acquired Agammaglobulinaemia Nos	Hypogammaglobulinaemia Nos
Hyper Igm

Recombinase Activating Gene 2 Deficiency

Common Variable Immunodeficiency

Cvid	Common Variable Agammaglobulinemia
Common Variable Immune Deficiency	Acquired Hypogammaglobulinemia
Hypogamma-Globulinemia, Acquired	Idiopathic Immunoglobulin Deficiency
Primary Antibody Deficiency	Primary Hypogammaglobulinemia
Acquired Agammaglobulinemia	Sporadic Hypogammaglobulinemia
Common Variable Hypogamma-Globulinemia	Immunoglobulin Deficiency, Late-Onset
Common Variable Hypogammaglobulinemia	Immunodeficiency, Common Variable

Severe Combined Immunodeficiency

Scid	Severe Combined Immunodeficiency Disease
Combined T And B Cell Inborn Immunodeficiency	Immunodeficiency, Severe Combined
Scid - [Severe Combined Immunodeficiencies]

Combined Immunodeficiency

Combined T Cell And B Cell Immunodeficiency	Congenital Combined Immunodeficiency
Syndrome With Combined Immunodeficiency	Combined T And B Cell Immunodeficiency
Combined Immunity Deficiency	Combined Immunodeficiency Syndrome
Combined T-Cell And B-Cell Immunodeficiency	Lymphopenic Agammaglobulinaemia

Recombinase Activating Gene 1 Deficiency

Severe Combined Immunodeficiency With Sensitivity To Ionizing Radiation

Severe Combined Immunodeficiency, Athabascan Type	Severe Combined Immunodeficiency Due To Dclre1c Deficiency
Rs-Scid	Scid, Autosomal Recessive, T Cell-Negative, B Cell-Negative, Nk Cell-Positive, With Sensitivity To Ionizing Radiation
Scid Due To Artemis Deficiency	Scid Due To Dclre1c Deficiency
Scid, Athabascan Type	Scid, Athabaskan Type
Severe Combined Immunodeficiency Due To Artemis Deficiency	Severe Combined Immunodeficiency, Athabaskan Type
SCIDA	Severe Combined Immunodeficiency, Athabascan-Type
Artemis Deficiency	Severe Combined Immunodeficiency Athabaskan Type
Severe Combined Immunodeficiency Autosomal Recessive T-Cell-Negative/B-Cell-Negative/Nk-Cell-Positive With Sensitivity To Ionizing Radiation	RSSCID
Athabascan Scid	Immunodeficiency, Severe Combined, Athabascan Type
Severe Combined Immunodeficiency, Athabaskan-Type

T Cell Deficiency

T Cell Immunodeficiency	T Lymphocyte Deficiency
T Lymphocyte Immunodeficiency	T-Lymphocyte Deficiency

Janus Kinase-3 Deficiency

Inflammatory Bowel Disease 15

IBD15

Osteopetrosis

Marble Bone Disease	Albers-Schonberg Disease
Osteopetroses	Marble Bones
Osteopetrosis And Related Disorders	Congenital Osteopetrosis
Marble Bone	Albers-Schoenberg Disease
Albers-Schonberg Osteopetrosis	Osteosclerosis Fragilis
Ivory Bones

Adenosine Deaminase Deficiency

Ada Deficiency	Ada-Scid
Severe Combined Immunodeficiency Due To Adenosine Deaminase Deficiency	Adenosine Deaminase Deficient Severe Combined Immunodeficiency
Scid Due To Ada Deficiency	Severe Combined Immunodeficiency Due To Ada Deficiency
Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-Negative, B Cell-Negative, Nk Cell-Negative, Due To Adenosine Deaminase Deficiency	Ada
Scid Due To Adenosine Deaminase Deficiency

Loeffler Syndrome

Loeffler'S Pneumonia	Loffler'S Syndrome
Simple Pulmonary Eosinophilia

Immunodeficiency With Hyper-Igm, Type 1

Immunodeficiency, X-Linked, With Hyper-Igm	Hyper Igm Syndrome
HIGM1	Xhim
Hyper-Igm Syndrome	Higm
Hyper-Igm Syndrome 1	Immunodeficiency 3
Imd3	Immunodeficiency With Hyper-Igm
Immunodeficiency With Hyper Igm Type 1	Ihis
X-Linked Hyper Igm Syndrome	Hyper-Igm Immunodeficiency, X-Linked
Hyper Igm Immunodeficiency, X-Linked	Hyper Igm Syndrome 1
X-Linked Immunodeficiency With Hyper-Igm 1	Immunodeficiency, With Hyper Igm
Immunodeficiency, With Hyper Igm, Type 1	Hyper-Igm Immunodeficiency Syndrome, Type 1
Hyperimmunoglobulin M Syndrome

Bare Lymphocyte Syndrome, Type Ii

Mhc Class Ii Deficiency	Bare Lymphocyte Syndrome
Major Histocompatibility Complex Class Ii Deficiency	Bare Lymphocyte Syndrome 2
Bare Lymphocyte Syndrome Type 2	Severe Combined Immunodeficiency, Hla Class Ii-Negative
Bare Lymphocyte Syndrome, Type Ii, Complementation Group C	Bare Lymphocyte Syndrome, Type Ii, Complementation Group D
Bare Lymphocyte Syndrome Type Ii	Scid, Hla Class Ii-Negative
Bare Lymphocyte Syndrome, Type Ii, Complementation Group A	Bare Lymphocyte Syndrome, Type Ii, Complementation Group B
Scid Due To Absent Class Ii Hla Antigens	Hla Class 1 Deficiency
Scid, Hla Class 2-Negative	Bls Type Ii
Bare Lymphocyte Syndrome Type 2, Complementation Group A	Bare Lymphocyte Syndrome Type 2, Complementation Group E
Severe Combined Immunodeficiency	Bls, Type Ii
Bls	Bare Lymphocyte Syndrome, Type Ii, Complementation Group E
Blsii	Bls Type 1
Bls 2	Scid Due To Absence Of Class Ii Hla Antigens
Severe Combined Immunodeficiency Due To Absent Class Ii Human Leukocyte Antigens	Immunodeficiency By Defective Expression Of Mhc Class Ii
BLS2	Bare Lymphocyte Syndrome Type Ii Complementation Group A
Bare Lymphocyte Syndrome Type Ii Complementation Group B	Bare Lymphocyte Syndrome Type Ii Complementation Group C
Bare Lymphocyte Syndrome Type Ii Complementation Group D	Bare Lymphocyte Syndrome Type Ii Complementation Group E
Bls Ii	Hereditary Mhc Class Ii Deficiency
Hla Class Ii Deficient Combined Immunodeficiency	Mhc-Ii Deficiency
Scid Hla Class Ii-Negative	Severe Combined Immunodeficiency Hla Class Ii-Negative
Bl-2	Immunodeficiency By Defective Expression Of Hla Class 2
Hla Class 2-Negative Severe Combined Immunodeficiency

Lig4 Syndrome

Dna Ligase Iv Deficiency	Ligase 4 Syndrome
LIG4S

Purine Nucleoside Phosphorylase Deficiency

Purine-Nucleoside Phosphorylase Deficiency	Pnp Deficiency
Nucleoside Phosphorylase Deficiency	Immunodeficiency Due To Purine Nucleoside Phosphorylase Deficiency
Deficiency Of Inosine Phosphorylase	Pnpase Deficiency
PNPD

B Cell Deficiency

Immunoglobulin Heavy Chain Deficiency	B Cell Deficiencies
Immunoglobulin Heavy Chain Deletion	Humoral Immune Defect

Tyrosinemia, Type I

Tyrosinemia Type I	Hepatorenal Tyrosinemia
Fumarylacetoacetase Deficiency	Fah Deficiency
TYRSN1	Fumarylacetoacetate Hydrolase Deficiency
Tyrosinemia Type 1	Tyrosinemia 1
Fumarylacetoacetase

Purine-Pyrimidine Metabolic Disorder

Inborn Errors Of Purine-Pyrimidine Metabolism

Disorder Of Purine Or Pyrimidine Metabolism

Sodoku Disease

Spirillosis	Sodoku
Spirillary Fever	Rat-Bite Fever
Rat-Bite Fever Due To Spirillum Minus	Spirillary Rat-Bite Fever
Sokosho

T-Cell Acute Lymphoblastic Leukemia

T-Cell Leukemia	Acute T Cell Leukemia
Precursor T Lymphoblastic Leukemia	Precursor T-Lymphoblastic Lymphoma/Leukemia
T Acute Lymphoblastic Leukemia	T-Cell Acute Lymphocytic Leukaemia
T-Cell Lymphoblastic Leukemia/Lymphoma	Leukemia T-Cell
Leukemia, T-Cell	Leukemia, Acute, Lymphoblastic, T-Cell
Leukemia, T-Cell Acute Lymphoblastic	Leukemia, Acute T-Cell
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma	Precursor T-Cell Lymphoblastic Lymphoma
Precursor T Cell Lymphoblastic Leukemia/Lymphoblastic Lymphoma

Systemic Lupus Erythematosus

Lupus Nephritis	SLE
Disseminated Lupus Erythematosus	Systemic Lupus Erythematosus, Susceptibility To
Lupus Erythematosus, Systemic	Lupus Nephritis, Susceptibility To
Libman-Sacks Disease	Systemic Lupus Erythematosus Susceptibility To
Sle - Lupus Erythematosus, Systemic	Le Syndrome
Lupus	Lupus Erythematosus Systemic
Lupus Erythematosus, Systemic, Susceptibility To	Lupus Vulgaris
Lupus Erythematosus, Discoid	Lupus Erythematosus
Systemic Lupus Erythematosus Nos	Sle - [Systemic Lupus Erythematosus]

Inflammatory Bowel Disease 18

IBD18

Colonic Benign Neoplasm

Colon Neoplasm	Colonic Mass
Colonic Tumor	Neoplasm Of Colon
Neoplasm Of The Colon	Colonic Neoplasms
Colon Cancer	Colon Carcinoma Nos
Colonic Cancer	Metastatic Colon Cancer Nos

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS11623	RAG2 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	RAG2	VGNC	VGNC:45327
Rattus norvegicus	RAG2	RGD	RGD:1305588
Bos taurus	RAG2	VGNC	VGNC:33694
Macaca mulatta	RAG2	VGNC	VGNC:76650
Mus musculus	RAG2	MGD	MGI:97849
Felis catus	RAG2	VGNC	VGNC:81952

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