Anti-BTD 抗体 (CAB6284)

抗体名:	Anti-BTD Antibody
抗体コード:	CAB6284
抗体サイズ:	20uL, 50uL, 100uL
申し込み:	WB IHC
反応性:	Human, Mouse, Rat
宿主種:	Rabbit
免疫原:	Recombinant fusion protein containing a sequence corresponding to amino acids 284-543 of human BTD (NP_000051.1).

申し込み:	WB IHC
推奨希釈:	WB 1:500 - 1:2000 IHC 1:100 - 1:200
反応性:	Human, Mouse, Rat
ポジティブサンプル:	Mouse liver, Mouse kidney

免疫原:	Recombinant fusion protein containing a sequence corresponding to amino acids 284-543 of human BTD (NP_000051.1).
精製方法:	Affinity purification
ストレージバッファ:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
アイソタイプ:	IgG
順序:	IQKA FAVA FGIN VLAA NVHH PVLG MTGS GIHT PLES FWYH DMEN PKSH LIIA QVAK NPVG LIGA ENAT GETD PSHS KFLK ILSG DPYC EKDA QEVH CDEA TKWN VNAP PTFH SEMM YDNF TLVP VWGK EGYL HVCS NGLC CYLL YERP TLSK ELYA LGVF DGLH TVHG TYYI QVCA LVRC GGLG FDTC GQEI TEAT GIFE FHLW GNFS TSYI FPLF LTSG MTLE VPDQ LGWE NDHY FLRK SRLS SGLV TAAL YGRL YERD
遺伝子ID:	686
Uniprot:	P43251
セルラーロケーション:	Secreted, extracellular space
計算された分子量:	58kDa/61kDa
観察された分子量:	61kDa

同義語:

BTD

バックグラウンド:

The protein encoded by this gene functions to recycle protein-bound biotin by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. The encoded protein has also been shown to have biotinyl transferase activity. Mutations in this gene are associated with biotinidase deficiency. Multiple transcript variants encoding different isoforms have been described.

UniProt Protein Function:	BTD: Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. Defects in BTD are the cause of biotinidase deficiency (BTD deficiency); also called late-onset multiple carboxylase deficiency. BTD deficiency is a juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis. BTD deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur. Belongs to the CN hydrolase family. BTD/VNN subfamily.
UniProt Protein Details:	Protein type:EC 3.5.1.12; Ligase; Secreted, signal peptide; Cofactor and Vitamin Metabolism - biotin; Hydrolase; Secreted Chromosomal Location of Human Ortholog: 3p25 Cellular Component: extracellular space; mitochondrial matrix; apical part of cell; extracellular region; nucleolus; perikaryon Molecular Function:biotinidase activity; biotin carboxylase activity Biological Process: epidermis development; central nervous system development; vitamin metabolic process; biotin metabolic process; water-soluble vitamin metabolic process Disease: Biotinidase Deficiency
NCBI Summary:	The protein encoded by this gene functions to recycle protein-bound biotin by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. The encoded protein has also been shown to have biotinyl transferase activity. Mutations in this gene are associated with biotinidase deficiency. Multiple transcript variants encoding different isoforms have been described. [provided by RefSeq, Aug 2013]
UniProt Code:	P43251
NCBI GenInfo Identifier:	226693503
NCBI Gene ID:	686
NCBI Accession:	P43251.2
UniProt Secondary Accession:	P43251,Q96EM9, A6NHF2, B2R865, B4DFX1, B4DLJ9, B7Z7C9 F8W1Q3,
UniProt Related Accession:	P43251
Molecular Weight:	543
NCBI Full Name:	Biotinidase
NCBI Synonym Full Names:	biotinidase
NCBI Official Symbol:	BTD
NCBI Protein Information:	biotinidase; biotinase
UniProt Protein Name:	Biotinidase
Protein Family:	Biotinidase
UniProt Gene Name:	BTD
UniProt Entry Name:	BTD_HUMAN

	Western blot analysis of extracts of various cell lines, using BTD antibody (CAB6284) at 1:1000 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 Enhanced Kit (RM00021). Exposure time: 90s.
	Immunohistochemistry of paraffin-embedded rat brain using BTD antibody (CAB6284) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human liver cancer using BTD antibody (CAB6284) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human prostate using BTD antibody (CAB6284) at dilution of 1:100 (40x lens).

Antibodies

Anti-BTD Antibody (CAB13955)

BTD Antibody (PACO08056)

Secondary Antibody

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