Mouse Anti-OSGEP (AA 1-335) Recombinant Antibody (9E3) (CBMAB-O0609-CQ)

Name: Mouse Anti-OSGEP (AA 1-335) Recombinant Antibody (9E3)
SKU: CBMAB-O0609-CQ
Rating: 5 (1 reviews)

See all OSGEP antibodies

Compare Online Inquiry

Datasheet

SDS

Request for COA

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

Basic Information

Host Animal

Mouse

Clone

9E3

Application

FC, IHC-P, WB

Immunogen

Recombinant protein fragment corresponding to amino acids 1-335 of human OSGEP(NP_060277) produced in E. coli

Specificity

Human

Antibody Isotype

IgG2a

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

Buffer

PBS, pH 7.3, 1% BSA, 50% glycerol

Preservative

0.02% Sodium Azide

Concentration

0.82 mg/mL

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 1-335

More Infomation

Target

Full Name

O-Sialoglycoprotein Endopeptidase

Introduction

OSGEP (O-Sialoglycoprotein Endopeptidase) is a Protein Coding gene. Diseases associated with OSGEP include Galloway-Mowat Syndrome 3 and Galloway-Mowat Syndrome. Among its related pathways are Gene Expression and tRNA processing. Gene Ontology (GO) annotations related to this gene include transferase activity, transferring acyl groups other than amino-acyl groups and transferase activity, transferring acyl groups.

Entrez Gene ID

55644

UniProt ID

Q9NPF4

Alternative Names

O-Sialoglycoprotein Endopeptidase; T(6)A37 Threonylcarbamoyladenosine Biosynthesis Protein OSGEP; TRNA Threonylcarbamoyladenosine Biosynthesis Protein OSGEP; N6-L-Threonylcarbamoyladenine Synthase; T(6)A Synthase; HOSGEP; GCPL1; Probable TRNA Threonylcarbamoyladenosine Biosynthesis Protein OSGEP; Probable TRNA N6-Adenosine Threonylcarbamoyltransferase

Function

Component of the EKC/KEOPS complex that is required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t6A37) in tRNAs that read codons beginning with adenine. The complex is probably involved in the transfer of the threonylcarbamoyl moiety of threonylcarbamoyl-AMP (TC-AMP) to the N6 group of A37. OSGEP likely plays a direct catalytic role in this reaction, but requires other protein(s) of the complex to fulfill this activity.

Biological Process

tRNA threonylcarbamoyladenosine modificationManual Assertion Based On ExperimentIDA:UniProtKB

Cellular Location

Cytoplasm
Nucleus

Involvement in disease

Galloway-Mowat syndrome 3 (GAMOS3):
A form of Galloway-Mowat syndrome, a severe renal-neurological disease characterized by early-onset nephrotic syndrome associated with microcephaly, central nervous system abnormalities, developmental delays, and a propensity for seizures. Brain anomalies include gyration defects ranging from lissencephaly to pachygyria and polymicrogyria, and cerebellar hypoplasia. Most patients show facial dysmorphism characterized by a small, narrow forehead, large/floppy ears, deep-set eyes, hypertelorism and micrognathia. Additional variable features are visual impairment and arachnodactyly. Most patients die in early childhood.

Tao, Y., Zhou, W., Chen, C., Zhang, Q., Liu, Z., Xia, P., ... & Li, C. (2024). O-sialoglycoprotein Endopeptidase (OSGEP) Suppresses Hepatic Ischemia-Reperfusion Injury-Induced Ferroptosis Through Modulating the MEK/ERK Signaling Pathway. Molecular Biotechnology, 1-16.

Esmaeilzadeh, E., Moradi, A., & Khorram Khorshid, H. R. (2023). Whole-exome sequencing revealed a novel homozygous missense variant in OSGEP gene: a case report of Galloway–Mowat syndrome in Iran. CEN Case Reports, 12(4), 374-377.

Xu, S., Hu, L., Yang, L., Wu, B., Cao, Y., Zhang, R., ... & Hu, L. (2022). Galloway–Mowat Syndrome Type 3 Caused by OSGEP Gene Variants: A Case Report and Literature Review. Frontiers in Pediatrics, 10, 899991.

Baker, T., Caylor, R., Wang, J., Kilpatrick, M., Batalis, N., Wolff, D., & Welsh, C. (2022). Neuropathologic Findings in Galloway-Mowat Syndrome 3 With a Novel OSGEP Variant. Journal of Neuropathology & Experimental Neurology, 81(11), 947-949.

Teng, H., Liang, C., Liang, D., Li, Z., & Wu, L. (2021). Novel variants in OSGEP leading to Galloway-Mowat syndrome by altering its subcellular localization. Clinica Chimica Acta, 523, 297-303.

Yang, Y., He, Y., Zhen, L., & Li, D. Z. (2019). Fetal phenotype of Galloway-Mowat syndrome 3 caused by a specific OSGEP variant. European Journal of Obstetrics and Gynecology and Reproductive Biology, 242, 182-184.

Domingo-Gallego, A., Furlano, M., Pybus, M., Barraca, D., Martínez, A. B., Mora Muñoz, E., ... & Ars, E. (2019). Novel homozygous OSGEP gene pathogenic variants in two unrelated patients with Galloway-Mowat syndrome: case report and review of the literature. BMC nephrology, 20, 1-8.

Ask a question We look forward to hearing from you.

0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Have you used Mouse Anti-OSGEP (AA 1-335) Recombinant Antibody (9E3)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon

$30 eGift Card
Submit a review

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.).

Online Inquiry

Request bulk or custom quote Second antibody and isotype control

Contact us

Tel: (USA)
(UK)
Fax:

Global Locations

Email:

Online Inquiry