Sign in or Register   Sign in or Register
  |  

Mouse Anti-ATP2C1 Recombinant Antibody (4G12) (CBMAB-A4035-YC)

Provided herein is a Mouse monoclonal antibody against Human ATPase Secretory Pathway Ca2+ Transporting 1. The antibody can be used for immunoassay techniques, such as ELISA, IHC, IHC-P, WB.
See all ATP2C1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
4G12
Antibody Isotype
IgG1
Application
ELISA, IHC, IHC-P, WB

Basic Information

Immunogen
Purified recombinant fragment of ATP2C1 expressed in E. Coli.
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
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!]

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

Target

Full Name
ATPase Secretory Pathway Ca2+ Transporting 1
Introduction
ATP2C1 belongs to the family of P-type cation transport ATPases. This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of calcium ions. Defects in this gene cause Hailey-Hailey disease, an autosomal dominant disorder.
Entrez Gene ID
Human27032
Monkey719281
UniProt ID
HumanP98194
MonkeyF7DC49
Alternative Names
ATPase Secretory Pathway Ca2+ Transporting 1; Calcium-Transporting ATPase Type 2C Member 1; ATPase, Ca++ Transporting, Type 2C, Member 1; Secretory Pathway Ca2+/Mn2+ ATPase 1; ATP-Dependent Ca(2+) Pump PMR1; ATPase 2C1; EC 3.6.3.8; Benign Chronic Pemphigu
Function
ATP-driven pump that supplies the Golgi apparatus with Ca2+ and Mn2+ ions, both essential cofactors for processing and trafficking of newly synthesized proteins in the secretory pathway (PubMed:16192278, PubMed:30923126, PubMed:21187401, PubMed:12707275, PubMed:20439740).
Within a catalytic cycle, acquires Ca2+ or Mn2+ ions on the cytoplasmic side of the membrane and delivers them to the lumenal side. The transfer of ions across the membrane is coupled to ATP hydrolysis and is associated with a transient phosphorylation that shifts the pump conformation from inward-facing to outward-facing state (PubMed:16192278, PubMed:16332677, PubMed:30923126).
Plays a primary role in the maintenance of Ca2+ homeostasis in the trans-Golgi compartment with a functional impact on Golgi and post-Golgi protein sorting as well as a structural impact on cisternae morphology (PubMed:20439740, PubMed:14632183).
Responsible for loading the Golgi stores with Ca2+ ions in keratinocytes, contributing to keratinocyte differentiation and epidermis integrity (PubMed:14632183, PubMed:10615129, PubMed:20439740).
Participates in Ca2+ and Mn2+ ions uptake into the Golgi store of hippocampal neurons and regulates protein trafficking required for neural polarity (By similarity).
May also play a role in the maintenance of Ca2+ and Mn2+ homeostasis and signaling in the cytosol while preventing cytotoxicity (PubMed:21187401).
Biological Process
Actin cytoskeleton reorganization Source: UniProtKB
Calcium-dependent cell-cell adhesion via plasma membrane cell adhesion molecules Source: UniProtKB
Calcium ion transmembrane transport Source: GO_Central
Calcium ion transport Source: UniProtKB
Cellular calcium ion homeostasis Source: UniProtKB
Cellular manganese ion homeostasis Source: UniProtKB
Epidermis development Source: UniProtKB
Golgi calcium ion homeostasis Source: UniProtKB
Golgi calcium ion transport Source: UniProtKB
Ion transmembrane transport Source: Reactome
Manganese ion transport Source: UniProtKB
Positive regulation of Golgi to plasma membrane protein transport Source: UniProtKB
Positive regulation of I-kappaB kinase/NF-kappaB signaling Source: UniProtKB
Trans-Golgi network membrane organization Source: UniProtKB
Cellular Location
Trans-Golgi network membrane; Golgi stack membrane. During neuron differentiation, shifts from juxtanuclear Golgi position to multiple Golgi structures distributed over the neural soma with a predominance in the apical dendritic trunk.
Involvement in disease
Hailey-Hailey disease (HHD): Autosomal dominant disorder characterized by persistent blisters and suprabasal cell separation (acantholysis) of the epidermis, due to impaired keratinocyte adhesion. Patients lacking all isoforms except isoform 2 have HHD.
Topology
Cytoplasmic: 1-70 aa
Helical: 71-91 aa
Lumenal: 92-104 aa
Helical: 105-123 aa
Cytoplasmic: 124-262 aa
Helical: 263-282 aa
Lumenal: 283-294 aa
Helical: 295-312 aa
Cytoplasmic: 313-699 aa
Helical: 700-719 aa
Lumenal: 720-729 aa
Helical: 730-750 aa
Cytoplasmic: 751-770 aa
Helical: 771-793 aa
Lumenal: 794-808 aa
Helical: 809-828 aa
Cytoplasmic: 829-841 aa
Helical: 842-860 aa
Lumenal: 861-875 aa
Helical: 876-896 aa
Cytoplasmic: 897-919 aa

Xiao, Z., Liu, Z. G., Yang, X. L. O., Yu, S. M., Zeng, J. R., & Li, C. M. (2021). Two Novel Variants and One Previously Reported Variant in the ATP2C1 Gene in Chinese Hailey-Hailey Disease Patients. Molecular Syndromology, 12(3), 148-153.

Bone, R. N., Tong, X., Weaver, S. A., Muralidharan, C., Krishnan, P., Kono, T., & Evans-Molina, C. (2021). Loss of Secretory Pathway Ca2+ ATPase (SPCA1) Impairs Insulin Secretion and Reduces Autophagy in the Pancreatic Islet. bioRxiv.

He, J., Liu, J., Huang, Y., Zhuo, Y., Chen, W., Duan, D., ... & Hu, Z. (2020). Olfactory mucosa mesenchymal stem cells alleviate cerebral ischemia/reperfusion injury via golgi apparatus secretory pathway Ca2+-ATPase Isoform1. Frontiers in Cell and Developmental Biology, 8, 1199.

Xiao, H., Huang, X., Xu, H., Chen, X., Xiong, W., Yang, Z., ... & Deng, H. (2019). A novel splice‐site mutation in the ATP2C1 gene of a Chinese family with Hailey‐Hailey disease. Journal of cellular biochemistry, 120(3), 3630-3636.

Jia, W. X., Zhang, W. L., Zhao, S. J., Li, W. R., Wu, Y. D., Ma, H. J., & Li, C. R. (2019). Three novel ATP2C1 mutations in Chinese patients with Hailey-Hailey disease. Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii, 36(6), 767.

Xia, Z., Wei, J., Li, Y., Wang, J., Li, W., Wang, K., ... & Wang, F. (2017). Zebrafish slc30a10 deficiency revealed a novel compensatory mechanism of Atp2c1 in maintaining manganese homeostasis. PLoS genetics, 13(7), e1006892.

Deng, H., & Xiao, H. (2017). The role of the ATP2C1 gene in Hailey–Hailey disease. Cellular and Molecular Life Sciences, 74(20), 3687-3696.

Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-ATP2C1 Recombinant Antibody (4G12)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
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.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare