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Rabbit Anti-CLN5 Recombinant Antibody (EPR12197(B)) (CBMAB-1386-CN)

This product is a rabbit antibody that recognizes CLN5 of human. The antibody EPR12197(B) can be used for immunoassay techniques such as: WB, IHC-P, IF.
See all CLN5 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
EPR12197(B)
Antibody Isotype
IgG
Application
WB, IHC-P, IF

Basic Information

Immunogen
Synthetic peptide within Human CLN5 aa. 150-250 (Cysteine residue).
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
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
50% Glycerol, 0.05% BSA
Preservative
0.01% 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.

Target

Full Name
CLN5, Intracellular Trafficking Protein
Introduction
This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. It regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane.
Entrez Gene ID
Human1203
Mouse211286
Rat306128
UniProt ID
HumanO75503
MouseQ3UMW8
RatA0A1S3ENP5
Alternative Names
NCL
Function
Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane (PubMed:22431521).
Biological Process
Brain development Source: UniProtKB
Glycosylation Source: UniProtKB
Lysosomal lumen acidification Source: UniProtKB
Lysosome organization Source: GO_Central
Neurogenesis Source: UniProtKB
Neuron maturation Source: UniProtKB
Positive regulation of GTP binding Source: UniProtKB
Protein catabolic process Source: UniProtKB
Retrograde transport, endosome to Golgi Source: UniProtKB
Signal peptide processing Source: UniProtKB
Cellular Location
Ceroid-lipofuscinosis neuronal protein 5, secreted form: Lysosome
Ceroid-lipofuscinosis neuronal protein 5: Membrane. An amphipathic anchor region facilitates its association with the membrane.
Involvement in disease
Ceroid lipofuscinosis, neuronal, 5 (CLN5):
A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 5 comprise mixed combinations of granular, curvilinear, and fingerprint profiles.
Topology
Cytoplasmic: 1-23
Helical: 24-41
Lumenal: 42-358
PTM
N-glycosylated with both high mannose and complex type sugars. Glycosylation is important for proper folding and trafficking to the lysosomes.
Ceroid-lipofuscinosis neuronal protein 5:
The type II membrane signal anchor is proteolytically cleaved to produce a mature form that is transported to the lysosomes (Ceroid-lipofuscinosis neuronal protein 5, secreted form) (PubMed:24038957, PubMed:20052765).
Can undergo proteolytic cleavage at the C-terminus, probably by a cysteine protease and may involve the removal of approximately 10-15 residues from the C-terminal end (PubMed:26342652).
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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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