Anti-ABCB7 抗体 (CAB14699)

抗体名:	Anti-ABCB7 Antibody
抗体コード:	CAB14699
抗体サイズ:	20uL, 50uL, 100uL
申し込み:	WB IHC IF
反応性:	Human, Mouse, Rat
宿主種:	Rabbit
免疫原:	Recombinant fusion protein containing a sequence corresponding to amino acids 503-753 of human ABCB7 (NP_004290.2).

申し込み:	WB IHC IF
推奨希釈:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
反応性:	Human, Mouse, Rat
ポジティブサンプル:	A-431, 293T, Mouse heart

免疫原:	Recombinant fusion protein containing a sequence corresponding to amino acids 503-753 of human ABCB7 (NP_004290.2).
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:	AIVG GSGS GKST IVRL LFRF YEPQ KGSI YLAG QNIQ DVSL ESLR RAVG VVPQ DAVL FHNT IYYN LLYG NISA SPEE VYAV AKLA GLHD AILR MPHG YDTQ VGER GLKL SGGE KQRV AIAR AILK DPPV ILYD EATS SLDS ITEE TILG AMKD VVKH RTSI FIAH RLST VVDA DEII VLDQ GKVA ERGT HHGL LANP HSIY SEMW HTQS SRVQ NHDN PKWE AKKE NISK EEER KKLQ EEIV NSVK GCGN CSC
遺伝子ID:	22
Uniprot:	O75027
セルラーロケーション:	Mitochondrion inner membrane, Multi-pass membrane protein
計算された分子量:	78kDa/82kDa
観察された分子量:	83kDa

同義語:

ABCB7, ABC7, ASAT, Atm1p, EST140535

バックグラウンド:

The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance as well as antigen presentation. This gene encodes a half-transporter involved in the transport of heme from the mitochondria to the cytosol. With iron/sulfur cluster precursors as its substrates, this protein may play a role in metal homeostasis. Mutations in this gene have been associated with mitochondrial iron accumulation and isodicentric (X)(q13) and sideroblastic anemia. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene.

UniProt Protein Function:	ABCB7: Could be involved in the transport of heme from the mitochondria to the cytosol. Plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins. Defects in ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT). ASAT is a recessive disorder characterized by an infantile to early childhood onset of nonprogressive cerebellar ataxia and mild anemia with hypochromia and microcytosis. Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Transporter; Membrane protein, integral; Transporter, ABC family; Mitochondrial; Membrane protein, multi-pass Chromosomal Location of Human Ortholog: Xq13.3 Cellular Component: integral to membrane; mitochondrial inner membrane; mitochondrion Molecular Function:ATP binding; ATPase activity, coupled to transmembrane movement of substances; heme transporter activity Biological Process: cellular iron ion homeostasis; heme transport; transmembrane transport; transport Disease: Anemia, Sideroblastic, And Spinocerebellar Ataxia
NCBI Summary:	The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance as well as antigen presentation. This gene encodes a half-transporter involved in the transport of heme from the mitochondria to the cytosol. With iron/sulfur cluster precursors as its substrates, this protein may play a role in metal homeostasis. Mutations in this gene have been associated with mitochondrial iron accumulation and isodicentric (X)(q13) and sideroblastic anemia. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Nov 2012]
UniProt Code:	O75027
NCBI GenInfo Identifier:	8928549
NCBI Gene ID:	22
NCBI Accession:	O75027.2
UniProt Secondary Accession:	O75027,O75345, Q5VWY7, Q5VWY8, Q9BRE1, Q9UND1, Q9UP01 G3XAC4,
UniProt Related Accession:	O75027
Molecular Weight:	78,160 Da
NCBI Full Name:	ATP-binding cassette sub-family B member 7, mitochondrial
NCBI Synonym Full Names:	ATP binding cassette subfamily B member 7
NCBI Official Symbol:	ABCB7
NCBI Official Synonym Symbols:	ABC7; ASAT; Atm1p; EST140535
NCBI Protein Information:	ATP-binding cassette sub-family B member 7, mitochondrial
UniProt Protein Name:	ATP-binding cassette sub-family B member 7, mitochondrial
UniProt Synonym Protein Names:	ATP-binding cassette transporter 7; ABC transporter 7 protein
Protein Family:	ABC transporter B family
UniProt Gene Name:	ABCB7
UniProt Entry Name:	ABCB7_HUMAN

	Western blot analysis of extracts of various cell lines, using ABCB7 antibody (CAB14699) 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 Basic Kit (RM00020). Exposure time: 10s.
	Immunohistochemistry of paraffin-embedded rat heart using ABCB7 antibody (CAB14699) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human gastric cancer using ABCB7 antibody (CAB14699) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse brain using ABCB7 antibody (CAB14699) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of HeLa cells using ABCB7 antibody (CAB14699) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

Antibodies

ABCB7 Antibody (PACO00353)

ABCB7 Antibody (PACO07520)

Secondary Antibody

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