Mouse Anti-PCSK1 Recombinant Antibody (2G12) (CBMAB-P1092-YC)

Name: Mouse Anti-PCSK1 Recombinant Antibody (2G12)
SKU: CBMAB-P1092-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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

Basic Information

Host Animal

Mouse

Clone

2G12

Application

ELISA, WB

Immunogen

PCSK1 (NP_000430, 652-753 aa) partial recombinant protein with GST tag. Immunogen sequence: GGRRDELEEG APSEAMLRLL QSAFSKNSPP KQSPKKSPTA KLNIPYENFY EALEKLNKPS QLKDSEDSLY NDYVDVFYNT KPYKHRDDRL LQALVDILNE EN

Specificity

Human

Antibody Isotype

IgG1, κ

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!]

Storage

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

Epitope

aa 652-753

More Infomation

Target

Full Name

proprotein convertase subtilisin/kexin type 1

Introduction

PCSK1 is a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. The encoded protein undergoes an initial autocatalytic processing event in the ER to generate a heterodimer which exits the ER and sorts to subcellular compartments where a second autocatalytic even takes place and the catalytic activity is acquired. The protease is packaged into and activated in dense core secretory granules and expressed in the neuroendocrine system and brain. This gene encodes one of the seven basic amino acid-specific members which cleave their substrates at single or paired basic residues. It functions in the proteolytic activation of polypeptide hormones and neuropeptides precursors. Mutations in this gene have been associated with susceptibility to obesity and proprotein convertase 1/3 deficiency. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene

Entrez Gene ID

5122

UniProt ID

P29120

Alternative Names

Proprotein Convertase Subtilisin/Kexin Type 1; Prohormone Convertase 1; Neuroendocrine Convertase 1; Prohormone Convertase 3; Proprotein Convertase 1; EC 3.4.21.93; NEC1;

Function

Involved in the processing of hormone and other protein precursors at sites comprised of pairs of basic amino acid residues. Substrates include POMC, renin, enkephalin, dynorphin, somatostatin, insulin and AGRP.

Biological Process

Cell-cell signalingManual Assertion Based On ExperimentTAS:ProtInc
NeurogenesisIEA:Ensembl
Pancreas developmentIEA:Ensembl
Peptide biosynthetic processISS:BHF-UCL
Peptide hormone processingManual Assertion Based On ExperimentIBA:GO_Central
Pituitary gland developmentIEA:Ensembl
Positive regulation of protein secretionIEA:Ensembl
Protein autoprocessingIEA:Ensembl
Protein processingManual Assertion Based On ExperimentIBA:GO_Central
ProteolysisManual Assertion Based On ExperimentTAS:ProtInc
Response to axon injuryIEA:Ensembl
Response to calcium ionIEA:Ensembl
Response to chlorateIEA:Ensembl
Response to fatty acidIEA:Ensembl
Response to glucocorticoidIEA:Ensembl
Response to glucoseIEA:Ensembl
Response to interleukin-1IEA:Ensembl
Response to lipopolysaccharideIEA:Ensembl
Response to morphineIEA:Ensembl
Response to nutrient levelsIEA:Ensembl
Response to peptide hormoneIEA:Ensembl
Response to xenobiotic stimulusIEA:Ensembl

Cellular Location

Cytoplasmic vesicle, secretory vesicle
Localized in the secretion granules.

Involvement in disease

Proprotein convertase 1 deficiency (PC1 deficiency):
Characterized by obesity, hypogonadism, hypoadrenalism, reactive hypoglycemia as well as marked small-intestinal absorptive dysfunction It is due to impaired processing of prohormones.

PTM

O-glycosylated.

Folon, L., Baron, M., Toussaint, B., Vaillant, E., Boissel, M., Scherrer, V., ... & Bonnefond, A. (2023). Contribution of heterozygous PCSK1 variants to obesity and implications for precision medicine: a case-control study. The Lancet Diabetes & Endocrinology, 11(3), 182-190.

Shah, B. P., Sleiman, P. M., Mc Donald, J., Moeller, I. H., & Kleyn, P. (2023). Functional characterization of all missense variants in LEPR, PCSK1, and POMC genes arising from single-nucleotide variants. Expert Review of Endocrinology & Metabolism, 18(2), 209-219.

Van Dijck, E., Beckers, S., Diels, S., Huybrechts, T., Verrijken, A., Van Hoorenbeeck, K., ... & Van Hul, W. (2022). Rare heterozygous PCSK1 variants in human obesity: the contribution of the p. Y181H variant and a literature review. Genes, 13(10), 1746.

Shakya, M., Martin, N. K., Arunagiri, A., Martin, M. G., Arvan, P., Low, M. J., & Lindberg, I. (2022). The G209R mutant mouse as a model for human PCSK1 polyendocrinopathy. Endocrinology, 163(5), bqac024.

Duclaux-Loras, R., Bourgeois, P., Lavrut, P. M., Charbit-Henrion, F., Bonniaud-Blot, P., Maudinas, R., ... & Fabre, A. (2021). A novel mutation of PCSK1 responsible for PC1/3 deficiency in two siblings. Clinics and Research in Hepatology and Gastroenterology, 45(6), 101640.

Aerts, L., Terry, N. A., Sainath, N. N., Torres, C., Martín, M. G., Ramos-Molina, B., & Creemers, J. W. (2021). Novel homozygous inactivating mutation in the PCSK1 gene in an infant with congenital malabsorptive diarrhea. Genes, 12(5), 710.

Muhsin, N. I., Bentley, L., Bai, Y., Goldsworthy, M., & Cox, R. D. (2020). A novel mutation in the mouse Pcsk1 gene showing obesity and diabetes. Mammalian Genome, 31, 17-29.

Chou, C. L., Chen, T. J., Lin, C. Y., Lee, S. W., Wang, S. C., Chu, S. S., & Yang, C. C. (2020). PCSK1 overexpression in rectal cancer correlates with poor response to preoperative chemoradiotherapy and prognosis. OncoTargets and therapy, 3141-3150.

Jarvela, T. S., Shakya, M., Bachor, T., White, A., Low, M. J., & Lindberg, I. (2019). Reduced stability and pH-dependent activity of a common obesity-linked PCSK1 polymorphism, N221D. Endocrinology, 160(11), 2630-2645.

Pépin, L., Colin, E., Tessarech, M., Rouleau, S., Bouhours-Nouet, N., Bonneau, D., & Coutant, R. (2019). A new case of PCSK1 pathogenic variant with congenital proprotein convertase 1/3 deficiency and literature review. The Journal of clinical endocrinology & metabolism, 104(4), 985-993.

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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)
Proteogenomics
Other Protocols

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