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Mouse Anti-SEC24D Recombinant Antibody (CBXS-2680) (CBMAB-S5432-CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-2680
Antibody Isotype
IgG1, κ
Application
ELISA, WB

Basic Information

Immunogen
Partial recombinant corresponding to aa935-1032 from SEC24D (NP_055637) with GST tag
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
Buffer
PBS, pH 7.2
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
SEC24 Homolog D, COPII Coat Complex Component
Introduction
The protein encoded by this gene is a member of the SEC24 subfamily of the SEC23/SEC24 family, which is involved in vesicle trafficking. The encoded protein has similarity to yeast Sec24p component of COPII. COPII is the coat protein complex responsible for vesicle budding from the ER. This gene product is implicated in the shaping of the vesicle, and also in cargo selection and concentration. Mutations in this gene have been associated with Cole-Carpenter syndrome, a disorder affecting bone formation, resulting in craniofacial malformations and bones that break easily. Alternative splicing results in multiple transcript variants encoding different isoforms.
Entrez Gene ID
9871
UniProt ID
O94855
Alternative Names
SEC24 Homolog D, COPII Coat Complex Component; SEC24-Related Protein D; SEC24 (S. Cerevisiae) Related Gene Family, Member D; SEC24 Family, Member D (S. Cerevisiae); SEC24 Related Gene Family, Member D; Protein Transport Protein Sec24D; SEC24 Family Member D; KIAA0755; CLCRP2;
Function
Component of the coat protein complex II (COPII) which promotes the formation of transport vesicles from the endoplasmic reticulum (ER). The coat has two main functions, the physical deformation of the endoplasmic reticulum membrane into vesicles and the selection of cargo molecules for their transport to the Golgi complex (PubMed:17499046, PubMed:20427317, PubMed:18843296).
Plays a central role in cargo selection within the COPII complex and together with SEC24C may have a different specificity compared to SEC24A and SEC24B (PubMed:17499046, PubMed:20427317, PubMed:18843296).
May more specifically package GPI-anchored proteins through the cargo receptor TMED10 (PubMed:20427317).
May also be specific for IxM motif-containing cargos like the SNAREs GOSR2 and STX5 (PubMed:18843296).
Biological Process
Biological Process COPII-coated vesicle cargo loadingManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process endoplasmic reticulum to Golgi vesicle-mediated transportManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process in utero embryonic developmentIEA:Ensembl
Biological Process intracellular protein transportIEA:InterPro
Cellular Location
Cytoplasmic vesicle, COPII-coated vesicle membrane
Endoplasmic reticulum membrane
Cytoplasm, cytosol
Involvement in disease
Cole-Carpenter syndrome 2 (CLCRP2):
A form of Cole-Carpenter syndrome, a disorder characterized by features of osteogenesis imperfecta such as bone deformities and severe bone fragility with frequent fractures, in association with craniosynostosis, ocular proptosis, hydrocephalus, growth failure and distinctive facial features. Craniofacial findings include marked frontal bossing, midface hypoplasia, and micrognathia. Despite the craniosynostosis and hydrocephalus, intellectual development is normal. CLCRP2 inheritance is autosomal recessive.
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For research use only. Not intended for any clinical use.

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