Product Name
Protein CLN8 (Cln8), Recombinant Protein
Full Product Name
Recombinant Mouse Protein CLN8 (Cln8)
Product Gene Name
Cln8 recombinant protein
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
1-288aa; full length protein
Sequence
MTPVSSHGLA ESIFDLDYAS WKIRSTLAVA GFVFYLGVFV VCHQLSSSLN ATYRSLAAKE KVFWNLAATR AVFGVQSTTA GLWALLGDPV LYADKALGQQ NWCWFHITTA TGFFFFENVA VHLSNLFFRT FDLFLVVHHL FAFLGFLGSA INLRAGHYLA MTTLLLEMST PFTCISWMLL KAGWSDSLFW KANQWLMIHM FHCRMILTYH MWWVCFCHWD ALTSSLHLPH WALFLFGLAL LTAVINPYWT HKKTQQLLHP VDWNFAQEEA KGSRQERTNG QVPRKKRL
3D Structure
ModBase 3D Structure for Q9QUK3
Host
Cell Free Expression
Form/Format
Liquid containing glycerol
Storage Buffer
Tris-based buffer, 50% glycerol.
Preparation and Storage
Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.
Repeated freezing and thawing is not recommended. Store working aliquots at 4 degree C for up to one week.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of Cln8 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Product Categories/Family for Cln8 recombinant protein
Transmembrane Protein
Application Notes for Cln8 recombinant protein
This is a recombinant transmembrane protein expressed in a cell-free expression system.
NCBI/Uniprot data below describe general gene information for Cln8. It may not necessarily be applicable to this product.
NCBI Accession #
NP_036130.1
[Other Products]
NCBI GenBank Nucleotide #
NM_012000.3
[Other Products]
UniProt Primary Accession #
Q9QUK3
[Other Products]
UniProt Related Accession #
Q9QUK3[Other Products]
Molecular Weight
33,109 Da
NCBI Official Full Name
protein CLN8
NCBI Official Synonym Full Names
ceroid-lipofuscinosis, neuronal 8
NCBI Official Symbol
Cln8??[Similar Products]
NCBI Official Synonym Symbols
mnd
??[Similar Products]
NCBI Protein Information
protein CLN8
UniProt Protein Name
Protein CLN8
UniProt Gene Name
Cln8??[Similar Products]
UniProt Entry Name
CLN8_MOUSE
UniProt Comments for Cln8
CLN8: Could play a role in cell proliferation during neuronal differentiation and in protection against cell death. Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 (CLN8). A form of neuronal ceroid lipofuscinosis with onset in childhood. 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 8 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 Northern epilepsy variant (CLN8NE). A form of neuronal ceroid lipofuscinosis clinically characterized by epilepsy that presents between 5 and 10 years of age with frequent tonic-clonic seizures followed by progressive mental retardation. Visual loss is not a prominent feature. Intracellular accumulation of autofluorescent material results in curvilinear and granular profiles on ultrastructural analysis.
Protein type: Membrane protein, multi-pass; Endoplasmic reticulum; Apoptosis; Membrane protein, integral; Cell development/differentiation
Cellular Component: endoplasmic reticulum; ER-Golgi intermediate compartment; integral to membrane; membrane
Biological Process: ***** locomotory behavior; ***** walking behavior; age-dependent response to oxidative stress; associative learning; cellular macromolecule catabolic process; cellular protein catabolic process; cellular protein metabolic process; ceramide metabolic process; cholesterol metabolic process; glutamate uptake during transmission of nerve impulse; lysosome organization and biogenesis; mitochondrial membrane organization and biogenesis; musculoskeletal movement; negative regulation of apoptosis; negative regulation of transferase activity; nervous system development; neurofilament cytoskeleton organization and biogenesis; neuromuscular process controlling balance; neuromuscular process controlling posture; phospholipid metabolic process; photoreceptor cell maintenance; regulation of cell size; retina development in camera-type eye; social behavior; somatic motor neuron differentiation; spinal cord motor neuron differentiation; visual perception
Disease: Ceroid Lipofuscinosis, Neuronal, 8
Research Articles on Cln8
1. This study demonistrated that Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways in neuronal ceroid lipofuscinosis 8 decifiency mice.
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
