CXCR4

C-X-C chemokine receptor type 4 (CXC-R4) (CXCR-4) (FB22) (Fusin) (HM89) (LCR1) (Leukocyte-derived seven transmembrane domain receptor) (LESTR) (Lipopolysaccharide-associated protein 3) (LAP-3) (LPS-associated protein 3) (NPYRL) (Stromal cell-derived factor 1 receptor) (SDF-1 receptor) (CD antigen CD184)

Homo sapiens (Human)

39746

352

Pending Verification

C-X-C chemokine receptor type 4 (CXC-R4) (CXCR-4) (FB22) (Fusin) (HM89) (LCR1) (Leukocyte-derived seven transmembrane domain receptor) (LESTR) (Lipopolysaccharide-associated protein 3) (LAP-3) (LPS-associated protein 3) (NPYRL) (Stromal cell-derived factor 1 receptor) (SDF-1 receptor) (CD antigen CD184)

WHIM syndrome (WHIMS) [MIM:193670]: Immunodeficiency disease characterized by neutropenia, hypogammaglobulinemia and extensive human papillomavirus (HPV) infection. Despite the peripheral neutropenia, bone marrow aspirates from affected individuals contain abundant mature myeloid cells, a condition termed myelokathexis. {ECO:0000269|PubMed:12692554, ECO:0000269|PubMed:15536153}. Note=The disease is caused by mutations affecting the gene represented in this entry.; Note=CXCR4 mutations play a role in the pathogenesis of Waldenstroem macroglobulinemia (WM) and influence disease presentation and outcome, as well as response to therapy. WM is a B-cell lymphoma characterized by accumulation of malignant lymphoplasmacytic cells in the bone marrow, lymph nodes and spleen, and hypersecretion of monoclonal immunoglobulin M (IgM). Excess IgM production results in serum hyperviscosity, tissue infiltration, and autoimmune-related pathology. {ECO:0000269|PubMed:24366360, ECO:0000269|PubMed:24553177}.

MUTAGEN 2 9 Missing: Reduced CXCL12 binding. Abolishes signaling. {ECO:0000269|PubMed:10825158}.; MUTAGEN 4 20 Missing: Reduced CXCL12 binding. Impaired signaling. Reduced coreceptor activity for HIV-1 isolates LAI and NDK. {ECO:0000269|PubMed:10825158}.; MUTAGEN 7 7 Y->A: Reduced coreceptor activity for HIV-1 isolates LAI and NDK. Greatly reduced coreceptor activity for HIV-1 isolates LAI and NDK; when associated with A-12. {ECO:0000269|PubMed:10825158, ECO:0000269|PubMed:12034737}.; MUTAGEN 7 7 Y->F: Sulfate incorporation greatly reduced; when associated with F-12 and F-21. Moderate reduction in sulfate incorporation; when associated with F-12 and A-18. No sulfate incorporation and binding SDF-1alpha greatly reduced; when associated with F-12; A-18 and F-21. {ECO:0000269|PubMed:10825158, ECO:0000269|PubMed:12034737}.; MUTAGEN 8 8 T->A: No effect on sulfate incorporation; when associated with A-9 and A-13. {ECO:0000269|PubMed:12034737}.; MUTAGEN 9 9 S->A: No effect on sulfate incorporation; when associated with A-8 and A-13. {ECO:0000269|PubMed:12034737}.; MUTAGEN 10 20 Missing: Reduced CXCL12 binding. No effect on signaling. {ECO:0000269|PubMed:10825158}.; MUTAGEN 11 11 N->A: Reduced molecular weight. Enhanced coreceptor activity on R5 HIV-1 isolate Envs. Slight further enhancement of coreceptor activity; when associated with A-13. {ECO:0000269|PubMed:10756055}.; MUTAGEN 12 12 Y->A: Greatly reduced coreceptor activity for HIV-1 isolates LAI and NDK; when associated with A-7. {ECO:0000269|PubMed:10825158, ECO:0000269|PubMed:12034737}.; MUTAGEN 12 12 Y->F: Sulfate incorporation greatly reduced; when associated with F-7 and F-21. Moderate reduction in sulfate incorporation; when associated with F-7 and A-18. No sulfate incorporation and binding SDF-1alpha greatly reduced; when associated with F-7; A-18 and F-21. {ECO:0000269|PubMed:10825158, ECO:0000269|PubMed:12034737}.; MUTAGEN 13 13 T->A: Enhanced coreceptor activity on R5 HIV-1 isolate Envs. No effect on sulfate incorporation; when associated with A-8 and A-9. {ECO:0000269|PubMed:10756055}.; MUTAGEN 14 15 EE->AA: Reduced CXCL12 binding. Reduced coreceptor activity for HIV-1 isolate NDK. {ECO:0000269|PubMed:10825158}.; MUTAGEN 18 18 S->A: Sulfate incorporation greatly reduced; when associated with F-21. Moderate reduction in sulfate incorporation; when associated with F-7 and F-12. No sulfate incorporation and binding SDF-1alpha greatly reduced; when associated with F-7; F-12; and F-21. {ECO:0000269|PubMed:12034737}.; MUTAGEN 21 21 Y->A: Reduced CXCL12 binding. Reduced coreceptor activity for HIV-1 isolates LAI and NDk. {ECO:0000269|PubMed:10825158, ECO:0000269|PubMed:12034737}.; MUTAGEN 21 21 Y->F: Sulfate incorporation greatly reduced; when associated with F-7 and F-12. Sulfate incorporation greatly reduced; when associated with A-18. No sulfate incorporation and binding SDF-1alpha greatly reduced; when associated with F-7; F-12 and A-18. {ECO:0000269|PubMed:10825158, ECO:0000269|PubMed:12034737}.; MUTAGEN 97 97 D->N: Reduced CXCL12 binding. Abolishes signaling. Markedly reduced coreceptor activity for HIV-1 isolate LAI. {ECO:0000269|PubMed:10825158}.; MUTAGEN 119 119 N->D: No reduction of agonist-induced G-protein activation. {ECO:0000269|PubMed:17197449}.; MUTAGEN 119 119 N->K: Loss of agonist-induced G-protein activation. {ECO:0000269|PubMed:17197449}.; MUTAGEN 119 119 N->S: Constitutive G-protein activation, with further activation induced by agonist. {ECO:0000269|PubMed:17197449}.; MUTAGEN 125 125 L->W: Increased thermostability.; MUTAGEN 133 133 D->N: No reduction of agonist-induced G-protein activation. {ECO:0000269|PubMed:17197449}.; MUTAGEN 134 134 R->A: Loss of agonist-induced G-protein activation. {ECO:0000269|PubMed:17197449}.; MUTAGEN 135 135 Y->A: No reduction of agonist-induced G-protein activation. {ECO:0000269|PubMed:17197449}.; MUTAGEN 171 171 D->N: Reduced coreceptor activity for HIV-1 isolate NDK. {ECO:0000269|PubMed:10825158}.; MUTAGEN 176 176 N->A: Enhanced coreceptor activity on R5 HIV-1 isolate Envs; when associated with A-11. {ECO:0000269|PubMed:10756055}.; MUTAGEN 183 183 R->A: Reduced coreceptor activity for several HIV-1 isolates. {ECO:0000269|PubMed:10074102}.; MUTAGEN 187 187 D->A: Reduced CXCL12 binding. Abolishes signaling. {ECO:0000269|PubMed:10825158}.; MUTAGEN 188 188 R->A: Reduced coreceptor activity for several HIV-1 isolates. {ECO:0000269|PubMed:10074102}.; MUTAGEN 193 193 D->A,S,N: Greatly reduced coreceptor activity for HIV-1 isolate NDK. Reduced coreceptor activity for several other HIV-1 isolates. {ECO:0000269|PubMed:10074102, ECO:0000269|PubMed:10825158}.; MUTAGEN 193 193 D->R: Abolishes coreceptor activity for HIV-1 isolate NDK. Reduced coreceptor activity for several other HIV-1 isolates. {ECO:0000269|PubMed:10074102, ECO:0000269|PubMed:10825158}.; MUTAGEN 240 240 T->P: Retains ligand-binding affinity but abolishes signaling.; MUTAGEN 262 262 D->A: Markedly reduced coreceptor activity for HIV-1 isolate LAI. {ECO:0000269|PubMed:10825158}.; MUTAGEN 268 268 E->A: Markedly reduced coreceptor activity for HIV-1 isolate NDK. Less effect for HIV-1 isolate LAI. {ECO:0000269|PubMed:10825158}.; MUTAGEN 288 288 E->Q: Reduced CXCL12 binding. Impaired signaling. Reduced coreceptor activity for HIV-1 isolate LAI. Enhanced coreceptor activity for HIV-1 isolate NDK. {ECO:0000269|PubMed:10825158}.; MUTAGEN 310 310 K->R: No effect on ubiquitination by RNF113A. {ECO:0000269|PubMed:28978524}.; MUTAGEN 324 324 S->A: Moderate degradation. About 60% reduction in binding ITCH and no ubiquitination nor protein degradation; when associated with A-325. {ECO:0000269|PubMed:19116316}.; MUTAGEN 324 324 S->D: Enhanced binding to ITCH. Enhanced binding to ITCH and greatly increased protein degradation; when associated with D-324. {ECO:0000269|PubMed:19116316}.; MUTAGEN 324 324 S->D: Enhanced binding to ITCH. Enhanced binding to ITCH and greatly increased protein degradation; when associated with D-325. {ECO:0000269|PubMed:19116316}.; MUTAGEN 325 325 S->A: Moderate degradation. About 60% reduction in binding ITCH and no ubiquitination nor protein degradation; when associated with A-324. {ECO:0000269|PubMed:19116316}.; MUTAGEN 330 330 S->A: No effect on binding to ITCH. {ECO:0000269|PubMed:19116316}.; MUTAGEN 331 331 K->R: Loss of ubiquitination by RNF113A. {ECO:0000269|PubMed:28978524}.

MEGISIYTSDNYTEEMGSGDYDSMKEPCFREENANFNKIFLPTIYSIIFLTGIVGNGLVILVMGYQKKLRSMTDKYRLHLSVADLLFVITLPFWAVDAVANWYFGNFLCKAVHVIYTVNLYSSVLILAFISLDRYLAIVHATNSQRPRKLLAEKVVYVGVWIPALLLTIPDFIFANVSEADDRYICDRFYPNDLWVVVFQFQHIMVGLILPGIVILSCYCIIISKLSHSKGHQKRKALKTTVILILAFFACWLPYYIGISIDSFILLEIIKQGCEFENTVHKWISITEALAFFHCCLNPILYAFLGAKFKTSAQHALTSVSRGSSLKILSKGKRGGHSSVSTESESSSFHSS

Receptor for the C-X-C chemokine CXCL12/SDF-1 that transduces a signal by increasing intracellular calcium ion levels and enhancing MAPK1/MAPK3 activation (PubMed:10452968, PubMed:28978524, PubMed:18799424, PubMed:24912431). Involved in the AKT signaling cascade (PubMed:24912431). Plays a role in regulation of cell migration, e.g. during wound healing (PubMed:28978524). Acts as a receptor for extracellular ubiquitin; leading to enhanced intracellular calcium ions and reduced cellular cAMP levels (PubMed:20228059). Binds bacterial lipopolysaccharide (LPS) et mediates LPS-induced inflammatory response, including TNF secretion by monocytes (PubMed:11276205). Involved in hematopoiesis and in cardiac ventricular septum formation. Also plays an essential role in vascularization of the gastrointestinal tract, probably by regulating vascular branching and/or remodeling processes in endothelial cells. Involved in cerebellar development. In the CNS, could mediate hippocampal-neuron survival (By similarity). {ECO:0000250|UniProtKB:P70658, ECO:0000269|PubMed:10074102, ECO:0000269|PubMed:10452968, ECO:0000269|PubMed:10644702, ECO:0000269|PubMed:10825158, ECO:0000269|PubMed:11276205, ECO:0000269|PubMed:17197449, ECO:0000269|PubMed:18799424, ECO:0000269|PubMed:20048153, ECO:0000269|PubMed:20228059, ECO:0000269|PubMed:20505072, ECO:0000269|PubMed:24912431, ECO:0000269|PubMed:28978524, ECO:0000269|PubMed:8752280, ECO:0000269|PubMed:8752281}.; (Microbial infection) Acts as a coreceptor (CD4 being the primary receptor) for human immunodeficiency virus-1/HIV-1 X4 isolates and as a primary receptor for some HIV-2 isolates. Promotes Env-mediated fusion of the virus (PubMed:8849450, PubMed:8929542, PubMed:9427609, PubMed:10074122, PubMed:10756055). {ECO:0000269|PubMed:10074122, ECO:0000269|PubMed:10756055, ECO:0000269|PubMed:8849450, ECO:0000269|PubMed:8929542, ECO:0000269|PubMed:9427609}.

Alternative sequence (7); Chain (1); Disulfide bond (2); Domain (2); Frameshift (1); Glycosylation (6); Motif (1); Natural variant (2); Region (1); Sequence conflict (1); Signal peptide (1); Topological domain (2); Transmembrane (1); Proposed to be involved in systemic lupus erythematosus (SLE) disease process. {ECO:0000269|PubMed:23956418}.; Expressed in spleen, lymph node, peripheral blood leukocytes, bone marrow, small intestine, stomach, appendix, lung and trachea. Expression was detected in NK cells, activated B-cells, NK-cell line but not in promyelocytic, B-, or T-cell lines. Expressed in monocytes. Isoform 3 is expressed at much lower level than isoform 1. {ECO:0000269|PubMed:11220635, ECO:0000269|PubMed:11698418, ECO:0000269|PubMed:11802771, ECO:0000269|PubMed:12242590, ECO:0000269|PubMed:23695528}.

C-X-C chemokine receptor type 4 (CXC-R4) (CXCR-4) (FB22) (Fusin) (HM89) (LCR1) (Leukocyte-derived seven transmembrane domain receptor) (LESTR) (Lipopolysaccharide-associated protein 3) (LAP-3) (LPS-associated protein 3) (NPYRL) (Stromal cell-derived factor 1 receptor) (SDF-1 receptor) (CD antigen CD184)

Recombinant

HEK293

Lyophilized

7.4-7.5

Sterile-filtered colorless solution

1mg/ml

Standard

PBS

SDS-PAGE

> 98%

RP-HPLC

-20°C

This bioreagent is stable at 4°C (short-term) and -70°C(long-term). After reconstitution, sample may be stored at 4°C for 2-7 days and below -18°C for future use.

Reconstitute in sterile distilled H2O to no less than 100ug/ml; dilute reconstituted stock further in other aqueous solutions if needed. Please review COA for lot-specific instructions. Final measurements should be determined by the end-user for optimal performance.