Mouse Anti-JAGN1 Recombinant Antibody (C-3) (CBMAB-0300-WJ)

Name: Mouse Anti-JAGN1 Recombinant Antibody (C-3)
SKU: CBMAB-0300-WJ
Rating: 5 (1 reviews)

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Datasheet

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

C-3

Application

WB, IP, IF, ELISA

Specificity

Mouse, Rat, Human

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

jagunal homolog 1

Introduction

JAGN1 (Jagunal Homolog 1) is a Protein Coding gene. The protein encoded is a transmembrane protein which functions in the early secretory pathway and is necessary for neutrophil differentiation and survival. Diseases associated with JAGN1 include Neutropenia, Severe Congenital, 6, Autosomal Recessive and Severe Congenital Neutropenia. Acts as a regulator of neutrophil function, probably via its role in vesicle-mediated transport: required for defense against fungal pathogens and for granulocyte.

Entrez Gene ID

Human	84522
Mouse	67767
Rat	502872

UniProt ID

Human	Q8N5M9
Mouse	Q5XKN4
Rat	Q4KM64

Alternative Names

SCN6; GL009

Function

Endoplasmic reticulum transmembrane protein involved in vesicle-mediated transport, which is required for neutrophil function. Required for vesicle-mediated transport; it is however unclear whether it is involved in early secretory pathway or intracellular protein transport. Acts as a regulator of neutrophil function, probably via its role in vesicle-mediated transport: required for defense against fungal pathogens and for granulocyte colony-stimulating factor (GM-CSF) signaling pathway; possibly by regulating glycosylation and/or targeting of proteins contributing to the viability and migration of neutrophils.

Biological Process

Cellular response to tunicamycinIEA:Ensembl
Defense response to fungusIEA:Ensembl
Endoplasmic reticulum organizationManual Assertion Based On ExperimentIBA:GO_Central
ExocytosisManual Assertion Based On ExperimentTAS:UniProtKB
Granulocyte colony-stimulating factor signaling pathwayManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of insulin secretion involved in cellular response to glucose stimulusIEA:Ensembl
Neutrophil differentiationManual Assertion Based On ExperimentIMP:UniProtKB
Neutrophil mediated immunityIEA:Ensembl
Neutrophil migrationIEA:Ensembl
Protein transportIEA:UniProtKB-KW
Vesicle-mediated transportManual Assertion Based On ExperimentIMP:UniProtKB

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

Neutropenia, severe congenital 6, autosomal recessive (SCN6):
A disorder of hematopoiesis characterized by maturation arrest of granulopoiesis at the level of promyelocytes with peripheral blood absolute neutrophil counts below 0.5 x 10(9)/l and early onset of severe bacterial infections.

Topology

Cytoplasmic: 1-39
Helical: 40-60
Lumenal: 61-71
Helical: 72-92
Cytoplasmic: 93-96
Helical: 97-117
Lumenal: 118-137
Helical: 138-158
Cytoplasmic: 159-183

Hojabri, M., Farsi, Y., Jamee, M., Abolhassani, H., Khani, H. H. K., Karimi, A., ... & Sharafian, S. (2023). JAGN1 mutation with distinct clinical features; two case reports and literature review. BMC pediatrics, 23(1), 1-7.

Khandagale, A., Holmlund, T., Entesarian, M., Nilsson, D., Kalwak, K., Klaudel‐Dreszler, M., ... & Fadeel, B. (2021). Severe congenital neutropenia‐associated JAGN1 mutations unleash a calpain‐dependent cell death programme in myeloid cells. British Journal of Haematology, 192(1), 200-211.

VanWinkle, P. E., Parish, F., Edwards, Y. J., & Sztul, E. (2020). JAGN1, tetraspanins, and Erv proteins: is common topology indicative of common function in cargo sorting?. American Journal of Physiology-Cell Physiology, 319(4), C667-C674.

Cifaldi, C., Serafinelli, J., Petricone, D., Brigida, I., Di Cesare, S., Di Matteo, G., ... & Finocchi, A. (2019). Next-generation sequencing reveals A JAGN1 mutation in a syndromic child with intermittent neutropenia. Journal of Pediatric Hematology/Oncology, 41(4), e266-e269.

Khandagale, A., Lazzaretto, B., Carlsson, G., Sundin, M., Shafeeq, S., Römling, U., & Fadeel, B. (2018). JAGN1 is required for fungal killing in neutrophil extracellular traps: implications for severe congenital neutropenia. Journal of Leukocyte Biology, 104(6), 1199-1213.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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