Mouse Anti-PANK2 Recombinant Antibody (3G4) (CBMAB-P0725-YC)

Name: Mouse Anti-PANK2 Recombinant Antibody (3G4)
SKU: CBMAB-P0725-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

3G4

Application

FC, ICC, IF, WB

Immunogen

Full length human recombinant protein of human PANK2 (NP_705902) produced in HEK293T cell

Specificity

Human

Antibody Isotype

IgG1

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

PBS, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide

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

pantothenate kinase 2

Introduction

PANK2 is a protein belonging to the pantothenate kinase family and is the only member of that family to be expressed in mitochondria. Pantothenate kinase is a key regulatory enzyme in the biosynthesis of coenzyme A (CoA) in bacteria and mammalian cells. It catalyzes the first committed step in the universal biosynthetic pathway leading to CoA and is itself subject to regulation through feedback inhibition by acyl CoA species. Mutations in this gene are associated with HARP syndrome and pantothenate kinase-associated neurodegeneration (PKAN), formerly Hallervorden-Spatz syndrome. Alternative splicing, involving the use of alternate first exons, results in multiple transcripts encoding different isoforms.

Entrez Gene ID

80025

UniProt ID

Q9BZ23

Alternative Names

Pantothenate Kinase 2; Hallervorden-Spatz Syndrome; Pantothenic Acid Kinase 2; EC 2.7.1.33; C20orf48; Neurodegeneration With Brain Iron Accumulation 1 (Hallervorden-Spatz Syndrome); Pantothenate Kinase 2, Mitochondrial;

Function

Isoform 1
Mitochondrial isoform that catalyzes the phosphorylation of pantothenate to generate 4'-phosphopantothenate in the first and rate-determining step of coenzyme A (CoA) synthesis (PubMed:15659606, PubMed:17825826, PubMed:17242360, PubMed:16272150).
Required for angiogenic activity of umbilical vein of endothelial cells (HUVEC) (PubMed:30221726).
Isoform 4
Cytoplasmic isoform that catalyzes the phosphorylation of pantothenate to generate 4'-phosphopantothenate in the first and rate-determining step of coenzyme A (CoA) synthesis.

Biological Process

Aerobic respirationIEA:Ensembl
AngiogenesisManual Assertion Based On ExperimentIMP:UniProtKB
Coenzyme A biosynthetic processManual Assertion Based On ExperimentIBA:GO_Central
Mitochondrion morphogenesisIEA:Ensembl
Pantothenate metabolic process1 PublicationNAS:ParkinsonsUK-UCL
Phosphorylation1 PublicationNAS:ParkinsonsUK-UCL
Regulation of bile acid metabolic processManual Assertion Based On ExperimentIMP:ParkinsonsUK-UCL
Regulation of fatty acid metabolic processManual Assertion Based On ExperimentIMP:ParkinsonsUK-UCL
Regulation of mitochondrial membrane potentialIEA:Ensembl
Regulation of triglyceride metabolic processManual Assertion Based On ExperimentIMP:ParkinsonsUK-UCL
Spermatid developmentIEA:Ensembl

Cellular Location

Isoform 1
Mitochondrion
Mitochondrion intermembrane space
Nucleus
Localizes predominantly to the mitochondria and to a lesser extent to the nucleus. Found in both the mitochondria and the nucleus throughout the cell cycle, with the exception of the G2/M phase when it is restricted to mitochdondria.
Isoform 2
Cytoplasm
Isoform 3
Cytoplasm
Isoform 4
Cytoplasm

Involvement in disease

Neurodegeneration with brain iron accumulation 1 (NBIA1):
Autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in first decade with slow progression or onset in second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.
Hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP):
Rare syndrome with many clinical similarities to PKAN.

PTM

Synthesized as a 62-kDa precursor which is proteolytically processed by the mitochondrial-processing peptidase (MPP) via a 59-kDa intermediate to yield the mature mitochondrial 48-kDa subunit.

Tao, Y., Zhao, C., Han, D., Wei, Y., Wang, L., Song, W., & Li, X. (2023). Typical pantothenate kinase-associated neurodegeneration caused by compound heterozygous mutations in PANK2 gene in a Chinese patient: A case report and literature review. Frontiers in Neurology, 14, 1170557.

Talaverón-Rey, M., Álvarez-Córdoba, M., Villalón-García, I., Povea-Cabello, S., Suárez-Rivero, J. M., Gómez-Fernández, D., ... & Sánchez-Alcázar, J. A. (2023). Alpha-lipoic acid supplementation corrects pathological alterations in cellular models of pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels. Orphanet journal of rare diseases, 18(1), 80.

Santambrogio, P., Cozzi, A., Di Meo, I., Cavestro, C., Vergara, C., Rodríguez-Pascau, L., ... & Levi, S. (2023). PPAR Gamma Agonist Leriglitazone Recovers Alterations Due to Pank2-Deficiency in HiPS-Derived Astrocytes. Pharmaceutics, 15(1), 202.

Li, W. B., Shen, N. X., Zhang, C., Xie, H. C., Li, Z. Y., Cao, L., ... & Song, X. W. (2022). Novel PANK2 mutations in patients with pantothenate kinase-associated neurodegeneration and the genotype–phenotype correlation. Frontiers in Aging Neuroscience, 14, 848919.

Yang, F., Wang, J., Yang, Z., Ren, Z., & Zeng, F. (2022). PANK2 p. A170fs： a novel pathogenetic mutation, compound with PANK2 p. R440P, causing pantothenate kinase Associated neurodegeneration in a Chinese family. International Journal of Neuroscience, 132(6), 582-588.

Álvarez-Córdoba, M., Reche-López, D., Cilleros-Holgado, P., Talaverón-Rey, M., Villalón-García, I., Povea-Cabello, S., ... & Sánchez-Alcázar, J. A. (2022). Therapeutic approach with commercial supplements for pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels. Orphanet journal of rare diseases, 17(1), 311.

Mignani, L., Zizioli, D., Khatri, D., Facchinello, N., Schiavone, M., De Palma, G., & Finazzi, D. (2022). Bi-Allelic Mutations in Zebrafish pank2 Gene Lead to Testicular Atrophy and Perturbed Behavior without Signs of Neurodegeneration. International Journal of Molecular Sciences, 23(21), 12914.

Pan, S., & Zhu, C. (2020). Atypical pantothenate kinase-associated neurodegeneration with PANK2 mutations: clinical description and a review of the literature. Neurocase, 26(3), 175-182.

Werning, M., Müllner, E. W., Mlynek, G., Dobretzberger, V., Djinovic‐Carugo, K., Baron, D. M., ... & Salzer, U. (2020). PKAN neurodegeneration and residual PANK2 activities in patient erythrocytes. Annals of Clinical and Translational Neurology, 7(8), 1340-1351.

Zhang, Y., Zhou, D., & Yang, T. (2019). Novel PANK2 mutation in a Chinese boy with PANK2-associated neurodegeneration: a case report and review of Chinese cases. Medicine, 98(4), e14122.

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

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