Anti-CLN5 항체 (CAB12886)

Antibody Name:	Anti-CLN5 Antibody
Antibody SKU:	CAB12886
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 96-407 of human CLN5 (NP_006484.1).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	Mouse heart, Rat heart

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 96-407 of human CLN5 (NP_006484.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	IPSR RHWP VPYK RFDF RPKP DPYC QAKY TFCP TGSP IPVM EGDD DIEV FRLQ APVW EFKY GDLL GHLK IMHD AIGF RSTL TGKN YTME WYEL FQLG NCTF PHLR PEMD APFW CNQG AACF FEGI DDVH WKEN GTLV QVAT ISGN MFNQ MAKW VKQD NETG IYYE TWNV KASP EKGA ETWF DSYD CSKF VLRT FNKL AEFG AEFK NIET NYTR IFLY SGEP TYLG NETS VFGP TGNK TLGL AIKR FYYP FKPH LPTK EFLL SLLQ IFDA VIVH KQFY LFYN FEYW FLPM KFPF IKIT YEEI PLPI RNKT LSGL
Gene ID:	1203
Uniprot:	O75503
Cellular Location:	Lysosome
Calculated MW:	41kDa
Observed MW:	41kDa

Synonyms:

CLN5, NCL

Background:

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. The primary defect in NCL disorders is thought to be associated with lysosomal storage function.

UniProt Protein Function:	CLN5: Defects in CLN5 are the cause of neuronal ceroid lipofuscinosis type 5 (CLN5); also known as Finnish variant late-infantile neuronal ceroid lipofuscinosis (vLINCL). 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. Belongs to the CLN5 family.
UniProt Protein Details:	Protein type:Membrane protein, integral Chromosomal Location of Human Ortholog: 13q22.3 Cellular Component: endoplasmic reticulum; Golgi apparatus; integral to membrane; lysosomal membrane; lysosome; perinuclear region of cytoplasm Molecular Function:mannose binding; protein binding Biological Process: brain development; lysosomal lumen acidification; neurogenesis; retrograde transport, endosome to Golgi; signal peptide processing Disease: Ceroid Lipofuscinosis, Neuronal, 5
NCBI Summary:	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. The primary defect in NCL disorders is thought to be associated with lysosomal storage function.[provided by RefSeq, Oct 2008]
UniProt Code:	O75503
NCBI GenInfo Identifier:	187608866
NCBI Gene ID:	1203
NCBI Accession:	O75503.2
UniProt Secondary Accession:	O75503,B3KQK7,
UniProt Related Accession:	O75503
Molecular Weight:	41kDa
NCBI Full Name:	Ceroid-lipofuscinosis neuronal protein 5
NCBI Synonym Full Names:	ceroid-lipofuscinosis, neuronal 5
NCBI Official Symbol:	CLN5
NCBI Official Synonym Symbols:	NCL
NCBI Protein Information:	ceroid-lipofuscinosis neuronal protein 5
UniProt Protein Name:	Ceroid-lipofuscinosis neuronal protein 5
Protein Family:	Cln5-like protein
UniProt Gene Name:	CLN5

	Western blot analysis of extracts of various cell lines, using CLN5 antibody (CAB12886) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
	Immunohistochemistry of paraffin-embedded human appendix using CLN5 antibody (CAB12886) at dilution of 1:150 (40x lens).

Antibodies

CLN5 Antibody (PACO02444)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)