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

This product is a mouse antibody that recognizes OSGEP (AA 1-335). The antibody 9E3 can be used for immunoassay techniques such as: FC, IHC-P, WB.
See all OSGEP antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
9E3
Antibody Isotype
IgG2a
Application
FC, IHC-P, WB

Basic Information

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

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.

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

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