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Invitrogen™ CLN3 Polyclonal Antibody
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Catalog No. PIPA5109553
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100 μL
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PIPA5109553 100 μL
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Catalog No. PIPA5109553 Supplier Invitrogen™ Supplier No. PA5109553
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Description

Rabbit Polyclonal Antibody

Immunogen sequence: MGGCAGSRRR FSDSEGEETV PEPRLPLLDH QGAHWKNAVG FWLLGLCNNF SYVVMLSAAH DILSHKRTSG NQSHVDPGPT PIPHNSSSRF DCNSVSTAAV LLADILPTLV IKLLAPLGLH LLPYSPRVLV SGICAAGSFV LVAFSHSVGT SLCGVVFASI SSGLGEVTFL SLTAFYPRAV ISWWSSGTGG AGLLGALSYL GLTQAGLSPQ QTLLSMLGIP ALLLASYFLL LTSPEAQDPG GEEEAESAAR QPLIRTEAPE SKPGSSSSLS LRERWTVFKG.

CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with 6 transmembrane domains. The CLN3 protein localizes to the lysosomal membrane and plays a role in lysosomal function. It may act as a chaperone involved in the folding and unfolding of other proteins, namely subunit C of the ATP synthase complex. Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood caused by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C. Batten disease is the most common form of a group of disorders known as neuronal ceroid lipofuscinoses (NCLs). Symptoms of Batten disease include progressive loss of vision, seizures, and psychomotor disturbances.
TRUSTED_SUSTAINABILITY

Specifications

Antigen CLN3
Applications ELISA, Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 2.91 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene CLN3
Gene Accession No. Q13286, Q61124
Gene Alias AI323623; batten disease protein; Battenin; BTS; ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease); ceroid-lipofuscinosis, neuronal 3; CLN 3; Cln3; CLN3, battenin; Cln3p; JNCL; MGC102840; Protein CLN3
Gene Symbols CLN3
Host Species Rabbit
Immunogen Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human CLN3 (NP_0010358971).
Purification Method Affinity Chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 1201, 12752, 293485
Target Species Human, Mouse, Rat
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG
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Safety and Handling

WARNING: Cancer - www.P65Warnings.ca.gov
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