Mouse Anti-AGTPBP1 Recombinant Antibody (V2-180246) (CBMAB-A1675-YC)

Name: Mouse Anti-AGTPBP1 Recombinant Antibody (V2-180246)
SKU: CBMAB-A1675-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

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

Basic Information

Host Animal

Mouse

Clone

V2-180246

Application

ELISA, WB

Immunogen

Partial recombinant corresponding to aa1087-1187 from AGTPBP1 (NP_056054) with GST tag.

Host Species

Mouse

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!]

Format

Liquid in PBS, pH 7.2

Buffer

PBS, pH 7.4

Preservative

None

Concentration

Batch dependent

Storage

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

More Infomation

Target

Full Name

ATP/GTP binding protein 1

Introduction

NNA1 is a zinc carboxypeptidase that contains nuclear localization signals and an ATP/GTP-binding motif that was initially cloned from regenerating spinal cord neurons of the mouse.

Entrez Gene ID

23287

UniProt ID

Q9UPW5

Alternative Names

ATP/GTP Binding Protein 1; Nervous System Nuclear Protein Induced By Axotomy Protein 1 Homolog; Tubulinyl-Tyr Carboxypeptidase; Cytosolic Carboxypeptidase 1; Tyrosine Carboxypeptidase; Carboxypeptidase-Tubulin; Soluble Carboxypeptidase;

Function

Metallocarboxypeptidase that mediates deglutamylation of target proteins. Catalyzes the deglutamylation of polyglutamate side chains generated by post-translational polyglutamylation in proteins such as tubulins. Also removes gene-encoded polyglutamates from the carboxy-terminus of target proteins such as MYLK. Acts as a long-chain deglutamylase and specifically shortens long polyglutamate chains, while it is not able to remove the branching point glutamate, a process catalyzed by AGBL5/CCP5.

Biological Process

Cerebellar Purkinje cell differentiation
C-terminal protein deglutamylation
Eye photoreceptor cell differentiation
Mitochondrion organization
Neuromuscular process
Olfactory bulb development
Protein deglutamylation
Protein side chain deglutamylation

Cellular Location

Cytoplasm; Cytosol; Nucleus; Mitochondrion. Localizes in both the cytoplasm and nuclei of interphase and dividing cells.

Involvement in disease

Neurodegeneration, childhood-onset, with cerebellar atrophy (CONDCA): An autosomal recessive disorder characterized by early onset of progressive neurodegeneration affecting the central and peripheral nervous systems. Clinical features include global developmental delay, impaired intellectual development, poor or absent speech, and motor abnormalities. Brain imaging shows cerebellar atrophy. Death in childhood may occur.

Türay, S., Eröz, R., & Başak, A. N. (2021). A novel pathogenic variant in the 3ʹ end of the AGTPBP1 gene gives rise to neurodegeneration without cerebellar atrophy: an expansion of the disease phenotype?. neurogenetics, 22(2), 127-132.

Kwak, H. J., Gil, M., Chae, H. S., Seok, J., Soundrarajan, N., Saha, S. K., ... & Cho, S. G. (2020). Expression of ATP/GTP Binding Protein 1 Has Prognostic Value for the Clinical Outcomes in Non-Small Cell Lung Carcinoma. Journal of Personalized Medicine, 10(4), 263.

Sheffer, R., Gur, M., Brooks, R., Salah, S., Daana, M., Fraenkel, N., ... & Harel, T. (2019). Biallelic variants in AGTPBP1, involved in tubulin deglutamylation, are associated with cerebellar degeneration and motor neuropathy. European Journal of Human Genetics, 27(9), 1419-1426.

Karakaya, M., Paketci, C., Altmueller, J., Thiele, H., Hoelker, I., Yis, U., & Wirth, B. (2019). Biallelic variant in AGTPBP1 causes infantile lower motor neuron degeneration and cerebellar atrophy. American Journal of Medical Genetics Part A, 179(8), 1580-1584.

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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)
Proteogenomic
Other Protocols

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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.).

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