Mouse Anti-MARCH6 Recombinant Antibody (CBYC3-105) (CBMAB-1791-YC)

Name: Mouse Anti-MARCH6 Recombinant Antibody (CBYC3-105)
SKU: CBMAB-1791-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

CBYC3-105

Application

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!]

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

membrane-associated ring finger (C3HC4) 6

Introduction

MARCH6 is a member of a family of membrane-associated E3 ubiquitin ligases containing RING-CH-type zinc finger motifs. MARCH6 is an important regulatory step in thyroid hormone signalling.

Entrez Gene ID

10299

UniProt ID

O60337

Alternative Names

3-Phosphoglyceric Phosphokinase

Function

E3 ubiquitin-protein ligase that promotes 'Lys-48'-linked ubiquitination of target proteins, leading to their proteasomal degradation (PubMed:15673284).

Promotes ubiquitination of DIO2, leading to its degradation (PubMed:19651899).

Promotes ubiquitination of SQLE, leading to its degradation (PubMed:24449766).

E3 ubiquitin ligases accept ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfer the ubiquitin to targeted substrates. May cooperate with UBE2G1 (PubMed:15673284).

Biological Process

Endoplasmic reticulum mannose trimming Source: Reactome
ERAD pathway Source: ParkinsonsUK-UCL
Proteasomal protein catabolic process Source: ParkinsonsUK-UCL
Proteasome-mediated ubiquitin-dependent protein catabolic process Source: UniProtKB
Protein K48-linked ubiquitination Source: UniProtKB
Protein ubiquitination Source: UniProtKB
Ubiquitin-dependent ERAD pathway Source: GO_Central

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

Epilepsy, familial adult myoclonic, 3 (FAME3):
A form of familial myoclonic epilepsy, a neurologic disorder characterized by cortical hand tremors, myoclonic jerks and occasional generalized or focal seizures with a non-progressive or very slowly progressive disease course. Usually, myoclonic tremor is the presenting symptom, characterized by tremulous finger movements and myoclonic jerks of the limbs increased by action and posture. In a minority of patients, seizures are the presenting symptom. Some patients exhibit mild cognitive impairment. FAME3 inheritance is autosomal dominant.

Topology

Cytoplasmic: 1-91
Helical: 92-112
Extracellular: 113-142
Helical: 143-163
Cytoplasmic: 164-283
Helical: 284-304
Extracellular: 305-336
Helical: 337-357
Cytoplasmic: 358-376
Helical: 377-397
Extracellular: 398-421
Helical: 422-442
Cytoplasmic: 443-480
Helical: 481-501
Extracellular: 502-519
Helical: 520-540
Cytoplasmic: 541-632
Helical: 633-653
Extracellular: 654-678
Helical: 679-699
Cytoplasmic: 700-721
Helical: 722-742
Extracellular: 743-764
Helical: 765-785
Cytoplasmic: 786-815
Helical: 816-836
Extracellular: 837-848
Helical: 849-869
Cytoplasmic: 870-910

PTM

Auto-ubiquitinated, which results in proteasomal degradation.

Liu, Y., Xu, S., Huang, Y., Liu, S., Xu, Z., Wei, M., & Liu, J. (2021). MARCH6 promotes Papillary Thyroid Cancer development by destabilizing DHX9. International Journal of Biological Sciences, 17(13), 3401.

Tan, J. M. E., van der Stoel, M. M., van den Berg, M., van Loon, N. M., Moeton, M., Scholl, E., ... & Zelcer, N. (2020). The MARCH6-SQLE axis controls endothelial cholesterol homeostasis and angiogenic sprouting. Cell reports, 32(5).

Scott, N. A., Sharpe, L. J., Capell-Hattam, I. M., Gullo, S. J., Luu, W., & Brown, A. J. (2020). The cholesterol synthesis enzyme lanosterol 14α-demethylase is post-translationally regulated by the E3 ubiquitin ligase MARCH6. Biochemical Journal, 477(2), 541-555.

Sharpe, L. J., Howe, V., Scott, N. A., Luu, W., Phan, L., Berk, J. M., ... & Brown, A. J. (2019). Cholesterol increases protein levels of the E3 ligase MARCH6 and thereby stimulates protein degradation. Journal of Biological Chemistry, 294(7), 2436-2448.

Florian, R. T., Kraft, F., Leitão, E., Kaya, S., Klebe, S., Magnin, E., ... & Depienne, C. (2019). Unstable TTTTA/TTTCA expansions in MARCH6 are associated with Familial Adult Myoclonic Epilepsy type 3. Nature communications, 10(1), 4919.

Coates, H. W., Chua, N. K., & Brown, A. J. (2019). Consulting prostate cancer cohort data uncovers transcriptional control: regulation of the MARCH6 gene. Biochimica et Biophysica Acta (BBA)-Molecular and Cell Biology of Lipids, 1864(11), 1656-1668.

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

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