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Anti-CLN6 (N258) Antibody

品牌：Antibodies

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规格：50µl

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Anti-CLN6 (N258) Antibody

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Name:	Anti-CLN6 (N258) Antibody See all CLN6 primary antibodies
Description:	Rabbit polyclonal antibody to CLN6 (N258)
Specificity:	CLN6 (N258) pAb detects endogenous levels of CLN6 protein.
Applications:	WB, IHC
Reactivity:	Human, Mouse, Rat
Immunogen:	Synthetic peptide, corresponding to amino acids 229-274 of Human CLN6.
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Molecular Weight:	~ 40 kDa
Purity:	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Product Form:	1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.

Involvement in Disease:	Ceroid lipofuscinosis, neuronal, 6: 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 6 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Ceroid lipofuscinosis, neuronal, 4A: An *****-onset 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. CLN4A has no visual involvement and is characterized by progressive myoclonic epilepsy.
Cellular locations:	Endoplasmic reticulum membrane. Endoplasmic reticulum.
Database Links:	Entrez Gene: 54982?Human Entrez Gene: 76524?Mouse Entrez Gene: 315746?Rat Omim: 606725?Human SwissProt: Q9NWW5?Human SwissProt: A0PJN2?Mouse
Synonyms:	Ceroid lipofuscinosis, neuronal 6, late infantile, variant Antibody CLN6 Antibody CLN6 protein Antibody FLJ20561 Antibody HsT18960 Antibody Nclf Antibody Protein CLN6 Antibody
Information:	Target information shown above is from the UniProt Consortium.