Rabbit Anti-Human CTSC (a.a. 365-463) Polyclonal antibody

Name: Rabbit Anti-Human CTSC (a.a. 365-463) Polyclonal antibody
SKU: DPABH-12866
Rating: 5 (1 reviews)

Rabbit Anti-Human CTSC (a.a. 365-463) Polyclonal antibody for IHC-P, IHC-F, IF

COA

SDS

Datasheet

Key features and details

Target: CTSC
Host: Rabbit
Application: IHC-P, IHC-F, IF
Species Reactivity: Rat, Human, Mouse, Chicken, Dog, Pig, Cow, Horse, Rabbit

Summary

Specifications

Antibody Isotype

IgG

Species Reactivity

Rat; Human; Mouse; Chicken; Dog; Pig; Cow; Horse; Rabbit

Immunogen

KLH conjugated synthetic peptide derived from human cathepsin C light chain: 365-463/463

Conjugate

Unconjugated

Applications

Application Notes

IHC-P: 1:100-500
IHC-F: 1:100-500
IF: 1:100-500

General Notes

This product (Catalog No. DPABH-12866) is a rabbit-derived IgG polyclonal antibody raised against human CTSC, targeting the amino acid region 365–463 of the protein. This antibody exhibits cross-reactivity with CTSC from rat, mouse, chicken, dog, pig, cow, horse, and rabbit. It has been affinity purified by Protein A. This gene encodes a member of the peptidase C1 family and lysosomal cysteine proteinase that appears to be a central coordinator for activation of many serine proteinases in cells of the immune system. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate heavy and light chains that form a disulfide-linked dimer. A portion of the propeptide acts as an intramolecular chaperone for the folding and stabilization of the mature enzyme. This enzyme requires chloride ions for activity and can degrade glucagon. Defects in the encoded protein have been shown to be a cause of Papillon-Lefevre syndrome, an autosomal recessive disorder characterized by palmoplantar keratosis and periodontitis.
DPABH-12866 is suitable for use in IHC-P (1:100–1:500), IHC-F (1:100–1:500), IF (immunofluorescence) (1:100–1:500) applications.
DPABH-12866 is supplied as a liquid formulation in 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. For long-term storage, keep at −20 °C. For short-term use, store at 2–8 °C. Avoid repeated freeze–thaw cycles to maintain antibody integrity and performance.

Target

Alternative Names

cathepsin C light chain; Dipeptidyl peptidase I; AI047818; CATC; Cathepsin C; Cathepsin J; CPPI; CTSC; Dipeptidyl peptidase 1; Dipeptidyl transferase; DPP I; DPPI; EC 3.4.14.1; HMS; JP; JPD; MGC126959; PALS; PLS; DPP-I; DPPI

Entrez Gene ID

1075

UniProt ID

P53634

Citations

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Documents

pdf COA

pdf SDS

pdf Datasheet

QA & Reviews

Customer Reviews

References

Pyogenic liver abscess and peritonitis due to Rhizopus oryzae in a child with Papillon-Lefevre syndrome

EUROPEAN JOURNAL OF PEDIATRICS

Authors: Dalgic, Buket; Bukulmez, Aysegul; Sari, Sinan

Abstract

Papillon-Lefevre syndrome (PLS) is an autosomal recessive disease that is characterized by symmetric palmoplantar keratodermatitis and severe periodontal destruction. Mutations in the cathepsin C gene (CTSC) have recently been detected in PLS. Immune dysregulation, due to a mutation in CTSC, increases the risk of pyogenic infections in PLS patients. A child with PLS is presented here with liver abscesses and peritonitis caused by Rhizopus oryzae. His liver abscess and peritonitis were cured with amphotericin B without surgical care. This is the first case in the literature liver abscess due to Rhizopus oryzae in a child with PLS.

Association of Frontotemporal Dementia GWAS Loci with Late-Onset Alzheimer's Disease in a Northern Han Chinese Population

JOURNAL OF ALZHEIMERS DISEASE

Authors: Tan, Chen-Chen; Wan, Yu; Tan, Meng-Shan; Zhang, Wei; Wang, Zi-Xuan; Sun, Fu-Rong; Miao, Dan; Tan, Lan; Yu, Jin-Tai

Abstract

Background: Both Alzheimer's disease (AD) and frontotemporal dementia (FTD) are a class of neurodegenerative diseases. Strong similarities in cerebrospinal fluid biomarker, imaging markers, and disease progression profiles suggest that some or most of the pathophysiology is shared between AD and FTD. A recent large genome-wide association study reported several single nucleotide polymorphisms (SNPs) at the RAB38, RAB38/CTSC, HLA-DRA/HLA-DRB5, and BTNL2 in association with FTD. Objective: To explore whether these SNPs are associated with AD risk. Methods: We conducted a case-control study to investigate the association of FTD-associated loci in 2338 Han Chinese subjects. Results: We observed significant differences in genotype distributions of rs302668 (p(c) = 0.025), rs9268877 (p(c) = 0.025), rs9268856 (p < 0.001), and rs1980493 (p(c) = 0.045) between cases and controls. The SNPs rs16913634 for RAB38/CTSC was unrelated to LOAD risk (p = 0.088). Conclusion: The SNPs rs302668 in RAB38, rs9268877 and rs9268856 polymorphism in HLA-DRA/HLA-DRB5, and rs1980493 polymorphism in BTNL2 might play a role in the susceptibility to late-onset AD in the Han Chinese population.