Co-chaperones of the Mammalian Endoplasmic Reticulum - The Networking of Chaperones by Co-chaperones - page 180

Table 9.1 BiP and its interaction partners in the mammalian ER

Function Protein (synonym) Related human

disease

OMIM Animal

model

First
reference

Hsp70-type
chaperone

BiP (Grp78,
HspA5)

Haemolytic
uraemic
syndrome

235400 Embryonic

lethality or
surfactant
deficiency

Haas and
Wabl ( 1983 )

Hsp40-type
co-chaper-
ones

ERj1 (Htj1,
DNAJC1)

Brightman
et al. ( 1995 )

ERj2 (Sec63,
ERdj2)

Polycystic liver
disease colorec-
tal cancer

174050 Embryonic

lethality

Skowronek
et al. ( 1999 )

ERj3 (ERdj3,
DnaJB11, HEDJ,
Dj9)

Bies et al.
( 1999 )

ERj4 (ERdj4,
DnaJB9, MDG1)

Postnatal
lethality
(surfactant
deficiency)

Shen et al.
( 2002 )

ERj5 (ERdj5,
DnaJC10, JPDI)

No
phenotype

Hosoda
et al. ( 2003 );
Cunnea et al.
(2003)

ERj6 (p58 IPK ,
DnaJC3, ERdj6)

Diabetic
mouse

Rutkowski
et al. ( 2007 )

ERj7 (Gng10,
DnaJC25, ERdj7)

Zahedi et al.
( 2009 )

Nucleotide
exchange
factors

Grp170 (ORP150,
HYOU1)

Embryonic
lethality

Lin et al.
( 1993 )

Sil1 (BAP) Marinesco-

Sj￶gren
syndrome

248800 Woozy

mouse

Chung et al.
( 2002 )

Additional
co-chaper-
ones

Sig-1R (sigma-1
receptor)

Hayashi and
Su 2007

HspA5BP1 (GBP) Oh-hashi

et al. ( 2003 )

Additional
chaperones

Grp94 (CaBP4,
ERp99, gp96,
endoplasmin)

Embryonic
lethality

Shiu et al.
( 1977 )

Calnexin (IP90,
p88)

Postnatal
lethality

Degen and
Williams
( 1991 )

Calreticulin
(CaBP3, ERp60)

Embryonic
lethality

Burns et al.
( 1992 )