CXCR4

Gene Synonyms:

Protein Names:

Organism:

Homo sapiens (Human)

Mass (kDa):

39746

Length (aa):

352

Sequence:

MEGISIYTSDNYTEEMGSGDYDSMKEPCFREENANFNKIFLPTIYSIIFLTGIVGNGLVILVMGYQKKLRSMTDKYRLHLSVADLLFVITLPFWAVDAVANWYFGNFLCKAVHVIYTVNLYSSVLILAFISLDRYLAIVHATNSQRPRKLLAEKVVYVGVWIPALLLTIPDFIFANVSEADDRYICDRFYPNDLWVVVFQFQHIMVGLILPGIVILSCYCIIISKLSHSKGHQKRKALKTTVILILAFFACWLPYYIGISIDSFILLEIIKQGCEFENTVHKWISITEALAFFHCCLNPILYAFLGAKFKTSAQHALTSVSRGSSLKILSKGKRGGHSSVSTESESSSFHSS

Proteomics (Proteome ID):

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)

Proteomics (Chromosome):

UP000005640

Mass Spectrometry:

N/A

Function [CC]:

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}.

Metal Binding:

N/A

Site:

N/A

Tissue Specificity:

Expressed in numerous tissues, such as peripheral blood leukocytes, spleen, thymus, spinal cord, heart, placenta, lung, liver, skeletal muscle, kidney, pancreas, cerebellum, cerebral cortex and medulla (in microglia as well as in astrocytes), brain microvascular, coronary artery and umbilical cord endothelial cells. Isoform 1 is predominant in all tissues tested. {ECO:0000269|PubMed:11276205}.

Disease:

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}.

Mutagenesis:

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}.

Name:

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)

Class:

Subcategory:

Recombinant

Source:

Species:

Human

Amino Acid Sequence:

Tag:

Format:

Lyophilized

Formulation:

Sterile-filtered colorless solution

Formulation Concentration:

1mg/ml

Buffer Volume:

Standard

Buffer Solution:

PBS

pH:

7.4-7.5

NaCl:

Null

EDTA:

Null

Purity:

> 98%

Determined:

SDS-PAGE

Stained:

Inquire

Validated:

RP-HPLC

Storage:

-20°C

Stability:

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.

Preparation:

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.